{"@context":{"@vocab":"https://cir.nii.ac.jp/schema/1.0/","rdfs":"http://www.w3.org/2000/01/rdf-schema#","dc":"http://purl.org/dc/elements/1.1/","dcterms":"http://purl.org/dc/terms/","foaf":"http://xmlns.com/foaf/0.1/","prism":"http://prismstandard.org/namespaces/basic/2.0/","cinii":"http://ci.nii.ac.jp/ns/1.0/","datacite":"https://schema.datacite.org/meta/kernel-4/","ndl":"http://ndl.go.jp/dcndl/terms/","jpcoar":"https://github.com/JPCOAR/schema/blob/master/2.0/"},"@id":"https://cir.nii.ac.jp/crid/1363101969009056512.json","@type":"Article","productIdentifier":[{"identifier":{"@type":"DOI","@value":"10.3389/fnins.2020.00194"}},{"identifier":{"@type":"URI","@value":"https://www.frontiersin.org/article/10.3389/fnins.2020.00194/full"}},{"identifier":{"@type":"PMID","@value":"32269505"}}],"resourceType":"学術雑誌論文(journal article)","dc:title":[{"@value":"Omics Approach to Axonal Dysfunction of Motor Neurons in Amyotrophic Lateral Sclerosis (ALS)"}],"description":[{"notation":[{"@value":"Amyotrophic lateral sclerosis (ALS) is an intractable adult-onset neurodegenerative disease that leads to the loss of upper and lower motor neurons (MNs). The long axons of MNs become damaged during the early stages of ALS. Genetic and pathological analyses of ALS patients have revealed dysfunction in the MN axon homeostasis. However, the molecular pathomechanism for the degeneration of axons in ALS has not been fully elucidated. This review provides an overview of the proposed axonal pathomechanisms in ALS, including those involving the neuronal cytoskeleton, cargo transport within axons, axonal energy supply, clearance of junk protein, neuromuscular junctions (NMJs), and aberrant axonal branching. To improve understanding of the global changes in axons, the review summarizes omics analyses of the axonal compartments of neurons"}]}],"creator":[{"@id":"https://cir.nii.ac.jp/crid/1420282801200722816","@type":"Researcher","personIdentifier":[{"@type":"KAKEN_RESEARCHERS","@value":"70451599"},{"@type":"NRID","@value":"1000070451599"},{"@type":"NRID","@value":"9000001587001"},{"@type":"NRID","@value":"9000014628578"},{"@type":"NRID","@value":"9000411000742"},{"@type":"NRID","@value":"9000243891025"},{"@type":"NRID","@value":"9000411521606"},{"@type":"NRID","@value":"9000411518310"},{"@type":"NRID","@value":"9000020427572"},{"@type":"NRID","@value":"9000414926784"},{"@type":"NRID","@value":"9000409198291"},{"@type":"NRID","@value":"9000409999896"},{"@type":"NRID","@value":"9000021715701"},{"@type":"NRID","@value":"9000021946694"},{"@type":"NRID","@value":"9000405872950"},{"@type":"NRID","@value":"9000257872685"},{"@type":"NRID","@value":"9000021884278"},{"@type":"NRID","@value":"9000021861096"},{"@type":"NRID","@value":"9000257905148"},{"@type":"NRID","@value":"9000411817223"},{"@type":"NRID","@value":"9000414805035"},{"@type":"NRID","@value":"9000345198218"},{"@type":"NRID","@value":"9000412665619"},{"@type":"NRID","@value":"9000261041306"},{"@type":"NRID","@value":"9000399498225"},{"@type":"RESEARCHMAP","@value":"https://researchmap.jp/NaokiSuzuki"}],"foaf:name":[{"@value":"Naoki Suzuki"}]},{"@id":"https://cir.nii.ac.jp/crid/1383101969009057546","@type":"Researcher","foaf:name":[{"@value":"Tetsuya Akiyama"}]},{"@id":"https://cir.nii.ac.jp/crid/1383101969009058052","@type":"Researcher","foaf:name":[{"@value":"Hitoshi Warita"}]},{"@id":"https://cir.nii.ac.jp/crid/1383101969009057029","@type":"Researcher","foaf:name":[{"@value":"Masashi Aoki"}]}],"publication":{"publicationIdentifier":[{"@type":"EISSN","@value":"1662453X"}],"prism:publicationName":[{"@value":"Frontiers in Neuroscience"}],"dc:publisher":[{"@value":"Frontiers Media SA"}],"prism:publicationDate":"2020-03-25","prism:volume":"14","prism:startingPage":"194"},"reviewed":"false","dcterms:accessRights":"http://purl.org/coar/access_right/c_abf2","dc:rights":["https://creativecommons.org/licenses/by/4.0/"],"url":[{"@id":"https://www.frontiersin.org/article/10.3389/fnins.2020.00194/full"}],"createdAt":"2020-03-25","modifiedAt":"2020-03-25","foaf:topic":[{"@id":"https://cir.nii.ac.jp/all?q=amyotrophic%20lateral%20sclerosis%20(ALS)","dc:title":"amyotrophic lateral sclerosis (ALS)"},{"@id":"https://cir.nii.ac.jp/all?q=axonal%20dysfunction","dc:title":"axonal dysfunction"},{"@id":"https://cir.nii.ac.jp/all?q=motor%20nerve%20organoid","dc:title":"motor nerve organoid"},{"@id":"https://cir.nii.ac.jp/all?q=Neurosciences.%20Biological%20psychiatry.%20Neuropsychiatry","dc:title":"Neurosciences. Biological psychiatry. Neuropsychiatry"},{"@id":"https://cir.nii.ac.jp/all?q=axon%20branching","dc:title":"axon branching"},{"@id":"https://cir.nii.ac.jp/all?q=local%20translation","dc:title":"local translation"},{"@id":"https://cir.nii.ac.jp/all?q=omics%20analysis","dc:title":"omics analysis"},{"@id":"https://cir.nii.ac.jp/all?q=RC321-571","dc:title":"RC321-571"},{"@id":"https://cir.nii.ac.jp/all?q=Neuroscience","dc:title":"Neuroscience"}],"project":[{"@id":"https://cir.nii.ac.jp/crid/1040000782000191616","@type":"Project","projectIdentifier":[{"@type":"KAKEN","@value":"18K07519"},{"@type":"JGN","@value":"JP18K07519"},{"@type":"URI","@value":"https://kaken.nii.ac.jp/grant/KAKENHI-PROJECT-18K07519/"}],"notation":[{"@language":"ja","@value":"ヒトiPS細胞由来神経オルガノイドを用いた筋萎縮性側索硬化症の軸索病態の解析"},{"@language":"en","@value":"Analysis of axonal pathomechanism in amyotrophic lateral sclerosis using human iPS cell-derived nerve organoid."}]}],"relatedProduct":[{"@id":"https://cir.nii.ac.jp/crid/1050001335857497728","@type":"Article","resourceType":"学術雑誌論文(journal article)","relationType":["references"],"jpcoar:relatedTitle":[{"@language":"en","@value":"Loss of  Sfpq  Causes Long-Gene Transcriptopathy in the Brain"}]},{"@id":"https://cir.nii.ac.jp/crid/1050564285802709888","@type":"Article","resourceType":"学術雑誌論文(journal article)","relationType":["references"],"jpcoar:relatedTitle":[{"@language":"en","@value":"Linear ubiquitination is involved in the pathogenesis of optineurin-associated amyotrophic lateral sclerosis"}]},{"@id":"https://cir.nii.ac.jp/crid/1360002217144824448","@type":"Article","resourceType":"学術雑誌論文(journal article)","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Motor Neuron-specific Disruption of Proteasomes, but Not Autophagy, Replicates Amyotrophic Lateral Sclerosis"}]},{"@id":"https://cir.nii.ac.jp/crid/1360002219689102080","@type":"Article","resourceType":"学術雑誌論文(journal article)","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Mitochondria‐associated membrane collapse is a common pathomechanism in\n                    <i>\n                      <scp>SIGMAR</scp>\n                      1\n                    </i>\n                    ‐ and\n                    <i>\n                      <scp>SOD</scp>\n                      1\n                    </i>\n                    ‐linked\n                    <scp>ALS</scp>"}]},{"@id":"https://cir.nii.ac.jp/crid/1360011142931326592","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Coactivator condensation at super-enhancers links phase separation and gene control"}]},{"@id":"https://cir.nii.ac.jp/crid/1360011143721648000","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Removing dysfunctional mitochondria from axons independent of mitophagy under pathophysiological conditions"}]},{"@id":"https://cir.nii.ac.jp/crid/1360011144046833024","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Slowing of axonal transport is a very early event in the toxicity of ALS–linked SOD1 mutants to motor neurons"}]},{"@id":"https://cir.nii.ac.jp/crid/1360011144779081856","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Gain and loss of function of ALS-related mutations of TARDBP (TDP-43) cause motor deficits in vivo"}]},{"@id":"https://cir.nii.ac.jp/crid/1360011144790641024","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"C9orf72-mediated ALS and FTD: multiple pathways to disease"}]},{"@id":"https://cir.nii.ac.jp/crid/1360011144846990848","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Spatiotemporal dynamics of molecular pathology in amyotrophic lateral sclerosis"}]},{"@id":"https://cir.nii.ac.jp/crid/1360011145180624896","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Atomic-resolution structure of the CAP-Gly domain of dynactin on polymeric microtubules determined by magic angle spinning NMR spectroscopy"}]},{"@id":"https://cir.nii.ac.jp/crid/1360011145623905536","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"<i>C9orf72</i>\n                    is required for proper macrophage and microglial function in mice"}]},{"@id":"https://cir.nii.ac.jp/crid/1360011146183869696","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Unraveling gene expression profiles in peripheral motor nerve from amyotrophic lateral sclerosis patients: insights into pathogenesis"}]},{"@id":"https://cir.nii.ac.jp/crid/1360011146487259648","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Motor neuron vulnerability and resistance in amyotrophic lateral sclerosis"}]},{"@id":"https://cir.nii.ac.jp/crid/1360283690829801472","@type":"Article","resourceType":"学術雑誌論文(journal article)","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Prion-like Properties of Pathological TDP-43 Aggregates from Diseased Brains"}]},{"@id":"https://cir.nii.ac.jp/crid/1360283691145450112","@type":"Article","resourceType":"学術雑誌論文(journal article)","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Generation of a Motor Nerve Organoid with Human Stem Cell-Derived Neurons"}]},{"@id":"https://cir.nii.ac.jp/crid/1360283691775165952","@type":"Article","resourceType":"学術雑誌論文(journal article)","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Mutations of optineurin in amyotrophic lateral sclerosis"}]},{"@id":"https://cir.nii.ac.jp/crid/1360283693515532544","@type":"Article","resourceType":"学術雑誌論文(journal article)","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Motor axonal excitability properties are strong predictors for survival in amyotrophic lateral sclerosis"}]},{"@id":"https://cir.nii.ac.jp/crid/1360285707370678016","@type":"Article","resourceType":"学術雑誌論文(journal article)","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Mitochondrial behavior during axon regeneration/degeneration in vivo"}]},{"@id":"https://cir.nii.ac.jp/crid/1360285708452586112","@type":"Article","resourceType":"学術雑誌論文(journal article)","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Modeling sporadic ALS in iPSC-derived motor neurons identifies a potential therapeutic agent"}]},{"@id":"https://cir.nii.ac.jp/crid/1360292619533337088","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"<i>C9ORF72</i>\n            repeat expansions in mice cause TDP-43 pathology, neuronal loss, and behavioral deficits"}]},{"@id":"https://cir.nii.ac.jp/crid/1360292619560808448","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Gene expression profile of spinal motor neurons in sporadic amyotrophic lateral sclerosis"}]},{"@id":"https://cir.nii.ac.jp/crid/1360292619709613952","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"SOD1 mutation is assosiated with accumulation of neurofilaments in amyotrophic lateral scelaries"}]},{"@id":"https://cir.nii.ac.jp/crid/1360292619748675840","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Real-Time Imaging of Translation on Single mRNA Transcripts in Live Cells"}]},{"@id":"https://cir.nii.ac.jp/crid/1360292619881559936","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Axonal transport deficits and neurodegenerative diseases"}]},{"@id":"https://cir.nii.ac.jp/crid/1360292619887532672","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"<i>NEK1</i>mutations in familial amyotrophic lateral sclerosis"}]},{"@id":"https://cir.nii.ac.jp/crid/1360292620300650624","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Axonal mRNA in Uninjured and Regenerating Cortical Mammalian Axons"}]},{"@id":"https://cir.nii.ac.jp/crid/1360292620885481984","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"<scp>mRNA</scp> Localization: An Orchestration of Assembly, Traffic and Synthesis"}]},{"@id":"https://cir.nii.ac.jp/crid/1360292621108642432","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Increased Axonal Ribosome Numbers Is an Early Event in the Pathogenesis of Amyotrophic Lateral Sclerosis"}]},{"@id":"https://cir.nii.ac.jp/crid/1360292621501228416","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Glutamate-induced RNA localization and translation in neurons"}]},{"@id":"https://cir.nii.ac.jp/crid/1360564063941245440","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Fine Structural Study of Neurofibrillary Changes in a Family with Amyotrophic Lateral Sclerosis"}]},{"@id":"https://cir.nii.ac.jp/crid/1360565166122132480","@type":"Article","resourceType":"学術雑誌論文(journal article)","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Establishment of In Vitro FUS-Associated Familial Amyotrophic Lateral Sclerosis Model Using Human Induced Pluripotent Stem Cells"}]},{"@id":"https://cir.nii.ac.jp/crid/1360565166763547136","@type":"Article","resourceType":"学術雑誌論文(journal article)","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Degeneration and impaired regeneration of gray matter oligodendrocytes in amyotrophic lateral sclerosis"}]},{"@id":"https://cir.nii.ac.jp/crid/1360565167097426304","@type":"Article","resourceType":"学術雑誌論文(journal article)","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"A Seeding Reaction Recapitulates Intracellular Formation of Sarkosyl-insoluble Transactivation Response Element (TAR) DNA-binding Protein-43 Inclusions"}]},{"@id":"https://cir.nii.ac.jp/crid/1360565167515093888","@type":"Article","resourceType":"学術雑誌論文(journal article)","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"ALS-associated mutations in FUS disrupt the axonal distribution and function of SMN"}]},{"@id":"https://cir.nii.ac.jp/crid/1360565168405167872","@type":"Article","resourceType":"学術雑誌論文(journal article)","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Loss-of-function mutations in the\n            <i>C9ORF72</i>\n            mouse ortholog cause fatal autoimmune disease"}]},{"@id":"https://cir.nii.ac.jp/crid/1360567182347959552","@type":"Article","resourceType":"学術雑誌論文(journal article)","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Comprehensive targeted next-generation sequencing in Japanese familial amyotrophic lateral sclerosis"}]},{"@id":"https://cir.nii.ac.jp/crid/1360567183429673600","@type":"Article","resourceType":"学術雑誌論文(journal article)","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Dysregulated protocadherin-pathway activity as an intrinsic defect in induced pluripotent stem cell–derived cortical interneurons from subjects with schizophrenia"}]},{"@id":"https://cir.nii.ac.jp/crid/1360574093561571072","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"A functional equivalent of endoplasmic reticulum and Golgi in axons for secretion of locally synthesized proteins"}]},{"@id":"https://cir.nii.ac.jp/crid/1360574094126078336","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Compartmentalized Microfluidic Platforms: The Unrivaled Breakthrough of<i>In Vitro</i>Tools for Neurobiological Research"}]},{"@id":"https://cir.nii.ac.jp/crid/1360574094517487616","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Progranulin is Neurotrophic In Vivo and Protects against a Mutant TDP-43 Induced Axonopathy"}]},{"@id":"https://cir.nii.ac.jp/crid/1360574094600181888","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"FUS-SMN Protein Interactions Link the Motor Neuron Diseases ALS and SMA"}]},{"@id":"https://cir.nii.ac.jp/crid/1360574094866295552","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Axonal Ion Channel Dysfunction in<i>C9orf72</i>Familial Amyotrophic Lateral Sclerosis"}]},{"@id":"https://cir.nii.ac.jp/crid/1360574095434434176","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"TALENs: a widely applicable technology for targeted genome editing"}]},{"@id":"https://cir.nii.ac.jp/crid/1360574095859237504","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Dynamic Axonal Translation in Developing and Mature Visual Circuits"}]},{"@id":"https://cir.nii.ac.jp/crid/1360574096395428736","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Label-Free Biomedical Imaging with High Sensitivity by Stimulated Raman Scattering Microscopy"}]},{"@id":"https://cir.nii.ac.jp/crid/1360579819983714304","@type":"Article","resourceType":"学術雑誌論文(journal article)","relationType":["isReferencedBy"],"jpcoar:relatedTitle":[{"@value":"Genetics of amyotrophic lateral sclerosis: seeking therapeutic targets in the era of gene therapy"}]},{"@id":"https://cir.nii.ac.jp/crid/1360846641732739968","@type":"Article","resourceType":"学術雑誌論文(journal article)","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Monitoring peripheral nerve degeneration in ALS by label-free stimulated Raman scattering imaging"}]},{"@id":"https://cir.nii.ac.jp/crid/1360846642459790336","@type":"Article","resourceType":"学術雑誌論文(journal article)","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Mislocated FUS is sufficient for gain-of-toxic-function amyotrophic lateral sclerosis phenotypes in mice"}]},{"@id":"https://cir.nii.ac.jp/crid/1360846643381865088","@type":"Article","resourceType":"学術雑誌論文(journal article)","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Mutations in the vesicular trafficking protein annexin A11 are associated with amyotrophic lateral sclerosis"}]},{"@id":"https://cir.nii.ac.jp/crid/1360855567871072000","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"TIA1 Mutations in Amyotrophic Lateral Sclerosis and Frontotemporal Dementia Promote Phase Separation and Alter Stress Granule Dynamics"}]},{"@id":"https://cir.nii.ac.jp/crid/1360855568486129152","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Non-nuclear Pool of Splicing Factor SFPQ Regulates Axonal Transcripts Required for Normal Motor Development"}]},{"@id":"https://cir.nii.ac.jp/crid/1360855568571296896","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Rapid Temporal Dynamics of Transcription, Protein Synthesis, and Secretion during Macrophage Activation"}]},{"@id":"https://cir.nii.ac.jp/crid/1360855568830719232","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Variants in KIF1A gene in dominant and sporadic forms of hereditary spastic paraparesis"}]},{"@id":"https://cir.nii.ac.jp/crid/1360855569167725824","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Fragile X Syndrome: Loss of Local mRNA Regulation Alters Synaptic Development and Function"}]},{"@id":"https://cir.nii.ac.jp/crid/1360855569400550144","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Neuronal activity enhances tau propagation and tau pathology in vivo"}]},{"@id":"https://cir.nii.ac.jp/crid/1360855569608036224","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Bridging biophysics and neurology: aberrant phase transitions in neurodegenerative disease"}]},{"@id":"https://cir.nii.ac.jp/crid/1360855569940051456","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Cell-to-Cell Transmission of Dipeptide Repeat Proteins Linked to C9orf72-ALS/FTD"}]},{"@id":"https://cir.nii.ac.jp/crid/1360855570192371840","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Kainic acid-induced early genes activation and neuronal death in the medial extended amygdala of rats"}]},{"@id":"https://cir.nii.ac.jp/crid/1360855570432719872","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Genetic Correction of SOD1 Mutant iPSCs Reveals ERK and JNK Activated AP1 as a Driver of Neurodegeneration in Amyotrophic Lateral Sclerosis"}]},{"@id":"https://cir.nii.ac.jp/crid/1360855570489908224","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"NG2+ CNS Glial Progenitors Remain Committed to the Oligodendrocyte Lineage in Postnatal Life and following Neurodegeneration"}]},{"@id":"https://cir.nii.ac.jp/crid/1360855570535101056","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Mutant dynactin in motor neuron disease"}]},{"@id":"https://cir.nii.ac.jp/crid/1360855570645562496","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Poly-dipeptides encoded by the\n                    <i>C9orf72</i>\n                    repeats bind nucleoli, impede RNA biogenesis, and kill cells"}]},{"@id":"https://cir.nii.ac.jp/crid/1360855571158365824","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Retrovirus-like Gag Protein Arc1 Binds RNA and Traffics across Synaptic Boutons"}]},{"@id":"https://cir.nii.ac.jp/crid/1361137043579862656","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"The Autistic Neuron: Troubled Translation?"}]},{"@id":"https://cir.nii.ac.jp/crid/1361137043971936384","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Mitochondrial abnormalities and disruption of the neuromuscular junction precede the clinical phenotype and motor neuron loss in hFUSWT transgenic mice"}]},{"@id":"https://cir.nii.ac.jp/crid/1361137044001960832","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Antisense Proline-Arginine RAN Dipeptides Linked to C9ORF72-ALS/FTD Form Toxic Nuclear Aggregates that Initiate In Vitro and In Vivo Neuronal Death"}]},{"@id":"https://cir.nii.ac.jp/crid/1361137044155534336","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Inhibiting p38 MAPK alpha rescues axonal retrograde transport defects in a mouse model of ALS"}]},{"@id":"https://cir.nii.ac.jp/crid/1361137044394915968","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Improved proteostasis in the secretory pathway rescues Alzheimer’s disease in the mouse"}]},{"@id":"https://cir.nii.ac.jp/crid/1361137044465156096","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Human C9ORF72 Hexanucleotide Expansion Reproduces RNA Foci and Dipeptide Repeat Proteins but Not Neurodegeneration in BAC Transgenic Mice"}]},{"@id":"https://cir.nii.ac.jp/crid/1361137044495317376","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Super-Resolution Microscopy Reveals Presynaptic Localization of the ALS/FTD Related Protein FUS in Hippocampal Neurons"}]},{"@id":"https://cir.nii.ac.jp/crid/1361137045613229056","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"The Peripheral Nervous System in Amyotrophic Lateral Sclerosis: Opportunities for Translational Research"}]},{"@id":"https://cir.nii.ac.jp/crid/1361137045615412736","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Retrograde Axonal Degeneration in Parkinson Disease"}]},{"@id":"https://cir.nii.ac.jp/crid/1361137046008967424","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"RNA Granules Hitchhike on Lysosomes for Long-Distance Transport, Using Annexin A11 as a Molecular Tether"}]},{"@id":"https://cir.nii.ac.jp/crid/1361137046110014080","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Modeling Motor Neuron Resilience in ALS Using Stem Cells"}]},{"@id":"https://cir.nii.ac.jp/crid/1361137046273689472","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Genome-wide Analyses Identify KIF5A as a Novel ALS Gene"}]},{"@id":"https://cir.nii.ac.jp/crid/1361412342635024768","@type":"Article","resourceType":"学術雑誌論文(journal article)","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Loss of ALS2/Alsin Exacerbates Motor Dysfunction in a SOD1H46R-Expressing Mouse ALS Model by Disturbing Endolysosomal Trafficking"}]},{"@id":"https://cir.nii.ac.jp/crid/1361412891684151040","@type":"Article","resourceType":"学術雑誌論文(journal article)","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Aberrant axon branching via Fos-B dysregulation in FUS-ALS motor neurons"}]},{"@id":"https://cir.nii.ac.jp/crid/1361418518543235456","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"TDP-43 and FUS in amyotrophic lateral sclerosis and frontotemporal dementia"}]},{"@id":"https://cir.nii.ac.jp/crid/1361418518693603712","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Mutations in the profilin 1 gene cause familial amyotrophic lateral sclerosis"}]},{"@id":"https://cir.nii.ac.jp/crid/1361418518785169920","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Exome Sequencing Reveals VCP Mutations as a Cause of Familial ALS"}]},{"@id":"https://cir.nii.ac.jp/crid/1361418520182396032","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Systematic mapping of fragile X granules in the mouse brain reveals a potential role for presynaptic FMRP in sensorimotor functions"}]},{"@id":"https://cir.nii.ac.jp/crid/1361418521037789952","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Axonal transcription factors signal retrogradely in lesioned peripheral nerve"}]},{"@id":"https://cir.nii.ac.jp/crid/1361418521351351936","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"The endoplasmic reticulum‐Golgi pathway is a target for translocation and aggregation of mutant superoxide dismutase linked to ALS"}]},{"@id":"https://cir.nii.ac.jp/crid/1361699993508192128","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Peripherin and Neurofilament Protein Coexist in Spinal Spheroids of Motor Neuron Disease"}]},{"@id":"https://cir.nii.ac.jp/crid/1361699993799758080","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"HDAC6 inhibition reverses axonal transport defects in motor neurons derived from FUS-ALS patients"}]},{"@id":"https://cir.nii.ac.jp/crid/1361699993963598080","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Recent advances in branching mechanisms underlying neuronal morphogenesis"}]},{"@id":"https://cir.nii.ac.jp/crid/1361699994109312640","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Impaired DNA damage response signaling by FUS-NLS mutations leads to neurodegeneration and FUS aggregate formation"}]},{"@id":"https://cir.nii.ac.jp/crid/1361699995177346688","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"ALS/FTD-Linked Mutation in FUS Suppresses Intra-axonal Protein Synthesis and Drives Disease Without Nuclear Loss-of-Function of FUS"}]},{"@id":"https://cir.nii.ac.jp/crid/1361699995526465792","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Dynamic neuromuscular remodeling precedes motor-unit loss in a mouse model of ALS"}]},{"@id":"https://cir.nii.ac.jp/crid/1361699995852281216","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"The multiple sclerosis drug fingolimod (FTY720) stimulates neuronal gene expression, axonal growth and regeneration"}]},{"@id":"https://cir.nii.ac.jp/crid/1361699996307944832","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Dysregulation of mRNA Localization and Translation in Genetic Disease"}]},{"@id":"https://cir.nii.ac.jp/crid/1361981468779840512","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Dynamics of Translation of Single mRNA Molecules In Vivo"}]},{"@id":"https://cir.nii.ac.jp/crid/1361981468950306688","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Neurofilament Light and Polyadenylated mRNA Levels Are Decreased in Amyotrophic Lateral Sclerosis Motor Neurons"}]},{"@id":"https://cir.nii.ac.jp/crid/1361981468967555328","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Lost in Transportation: Nucleocytoplasmic Transport Defects in ALS and Other Neurodegenerative Diseases"}]},{"@id":"https://cir.nii.ac.jp/crid/1361981469155173376","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Gain of Toxicity from ALS/FTD-Linked Repeat Expansions in C9ORF72 Is Alleviated by Antisense Oligonucleotides Targeting GGGGCC-Containing RNAs"}]},{"@id":"https://cir.nii.ac.jp/crid/1361981469348976512","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"FALS with FUS mutation in Japan, with early onset, rapid progress and basophilic inclusion"}]},{"@id":"https://cir.nii.ac.jp/crid/1361981469662283392","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"A novel ALS-associated variant in UBQLN4 regulates motor axon morphogenesis"}]},{"@id":"https://cir.nii.ac.jp/crid/1361981469856815104","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Transmembrane Receptor DCC Associates with Protein Synthesis Machinery and Regulates Translation"}]},{"@id":"https://cir.nii.ac.jp/crid/1361981469984393216","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"<i>C9orf72</i>\n                    repeat expansions cause neurodegeneration in\n                    <i>Drosophila</i>\n                    through arginine-rich proteins"}]},{"@id":"https://cir.nii.ac.jp/crid/1361981470313851136","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Peripheral neuropathy via mutant tRNA synthetases: Inhibition of protein translation provides a possible explanation"}]},{"@id":"https://cir.nii.ac.jp/crid/1361981470346008960","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Evidence of kinesin heavy chain (\n            <i>KIF5A</i>\n            ) involvement in pure hereditary spastic paraplegia"}]},{"@id":"https://cir.nii.ac.jp/crid/1361981470491072896","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Amyotrophic lateral sclerosis is a distal axonopathy: evidence in mice and man"}]},{"@id":"https://cir.nii.ac.jp/crid/1361981470661262848","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Amyotrophic Lateral Sclerosis"}]},{"@id":"https://cir.nii.ac.jp/crid/1361981470780786560","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Axonal Transport: Cargo-Specific Mechanisms of Motility and Regulation"}]},{"@id":"https://cir.nii.ac.jp/crid/1361981470893167104","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Superoxide dismutase 1 with mutations linked to familial amyotrophic lateral sclerosis possesses significant activity."}]},{"@id":"https://cir.nii.ac.jp/crid/1361981471369699072","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Genetic validation of a therapeutic target in a mouse model of ALS"}]},{"@id":"https://cir.nii.ac.jp/crid/1362262944075601408","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Altered neuronal migratory trajectories in human cerebral organoids derived from individuals with neuronal heterotopia"}]},{"@id":"https://cir.nii.ac.jp/crid/1362262944197156864","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Molecular pathways of motor neuron injury in amyotrophic lateral sclerosis"}]},{"@id":"https://cir.nii.ac.jp/crid/1362262944255705472","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Genetics of Amyotrophic Lateral Sclerosis"}]},{"@id":"https://cir.nii.ac.jp/crid/1362262944278983808","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Subcellular transcriptome alterations in a cell culture model of spinal muscular atrophy point to widespread defects in axonal growth and presynaptic differentiation"}]},{"@id":"https://cir.nii.ac.jp/crid/1362262944389045248","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"NEK1 Mutations Cause Short-Rib Polydactyly Syndrome Type Majewski"}]},{"@id":"https://cir.nii.ac.jp/crid/1362262944435918848","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Translation dynamics of single mRNAs in live cells and neurons"}]},{"@id":"https://cir.nii.ac.jp/crid/1362262944541214208","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Real-time quantification of single RNA translation dynamics in living cells"}]},{"@id":"https://cir.nii.ac.jp/crid/1362262944866308864","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"∆FosB differentially modulates nucleus accumbens direct and indirect pathway function"}]},{"@id":"https://cir.nii.ac.jp/crid/1362262945348088064","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Axonally Synthesized ATF4 Transmits a Neurodegenerative Signal across Brain Regions"}]},{"@id":"https://cir.nii.ac.jp/crid/1362262945890893952","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Differential roles of α-, β-, and γ-actin in axon growth and collateral branch formation in motoneurons"}]},{"@id":"https://cir.nii.ac.jp/crid/1362262946136027904","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"A Functional Role for Intra-Axonal Protein Synthesis during Axonal Regeneration from Adult Sensory Neurons"}]},{"@id":"https://cir.nii.ac.jp/crid/1362262946352716544","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Hot-spot KIF5A mutations cause familial ALS"}]},{"@id":"https://cir.nii.ac.jp/crid/1362544418345569408","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Axonal transport defects: a common theme in neurodegenerative diseases"}]},{"@id":"https://cir.nii.ac.jp/crid/1362544418596445312","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Facilitation of axon regeneration by enhancing mitochondrial transport and rescuing energy deficits"}]},{"@id":"https://cir.nii.ac.jp/crid/1362544419163634816","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Astrocytes as determinants of disease progression in inherited amyotrophic lateral sclerosis"}]},{"@id":"https://cir.nii.ac.jp/crid/1362544419445733504","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Expression of ALS/FTD-linked mutant CCNF in zebrafish leads to increased cell death in the spinal cord and an aberrant motor phenotype"}]},{"@id":"https://cir.nii.ac.jp/crid/1362544419545435392","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"ALS-associated mutant FUS induces selective motor neuron degeneration through toxic gain of function"}]},{"@id":"https://cir.nii.ac.jp/crid/1362544419550071040","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Autism-like phenotype and risk gene mRNA deadenylation by CPEB4 mis-splicing"}]},{"@id":"https://cir.nii.ac.jp/crid/1362544419572331520","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Effect of spastic paraplegia mutations in KIF5A kinesin on transport activity"}]},{"@id":"https://cir.nii.ac.jp/crid/1362544419651721216","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Premature polyadenylation-mediated loss of stathmin-2 is a hallmark of TDP-43-dependent neurodegeneration"}]},{"@id":"https://cir.nii.ac.jp/crid/1362544419685407744","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"The FXG: A Presynaptic Fragile X Granule Expressed in a Subset of Developing Brain Circuits"}]},{"@id":"https://cir.nii.ac.jp/crid/1362544419711158016","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Non–cell autonomous effect of glia on motor neurons in an embryonic stem cell–based ALS model"}]},{"@id":"https://cir.nii.ac.jp/crid/1362544419867699712","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Loss and gain of FUS function impair neuromuscular synaptic transmission in a genetic model of ALS"}]},{"@id":"https://cir.nii.ac.jp/crid/1362544419932384256","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Semaphorin 3A Signaling Through Neuropilin-1 Is an Early Trigger for Distal Axonopathy in the SOD1<sup>G93A</sup>Mouse Model of Amyotrophic Lateral Sclerosis"}]},{"@id":"https://cir.nii.ac.jp/crid/1362544419940429312","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Intra‐axonal protein synthesis in development and beyond"}]},{"@id":"https://cir.nii.ac.jp/crid/1362544420652949760","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Endolysosomal Deficits Augment Mitochondria Pathology in Spinal Motor Neurons of Asymptomatic fALS Mice"}]},{"@id":"https://cir.nii.ac.jp/crid/1362544420749859328","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Nuclear-Import Receptors Reverse Aberrant Phase Transitions of RNA-Binding Proteins with Prion-like Domains"}]},{"@id":"https://cir.nii.ac.jp/crid/1362544420827134592","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Mild ALS in Japan associated with novel SOD mutation"}]},{"@id":"https://cir.nii.ac.jp/crid/1362825893281688064","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Mutant Profilin1 transgenic mice recapitulate cardinal features of motor neuron disease"}]},{"@id":"https://cir.nii.ac.jp/crid/1362825893581792768","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Genetic mutations in RNA-binding proteins and their roles in ALS"}]},{"@id":"https://cir.nii.ac.jp/crid/1362825893653749120","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Muscle Nogo‐a expression is a prognostic marker in lower motor neuron syndromes"}]},{"@id":"https://cir.nii.ac.jp/crid/1362825893663379456","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Altered axonal excitability properties in amyotrophic lateral sclerosis: impaired potassium channel function related to disease stage"}]},{"@id":"https://cir.nii.ac.jp/crid/1362825894085304704","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"A Hexanucleotide Repeat Expansion in C9ORF72 Is the Cause of Chromosome 9p21-Linked ALS-FTD"}]},{"@id":"https://cir.nii.ac.jp/crid/1362825894241850368","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"TDP-43 repression of nonconserved cryptic exons is compromised in ALS-FTD"}]},{"@id":"https://cir.nii.ac.jp/crid/1362825894334684288","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Fusion of Regionally Specified hPSC-Derived Organoids Models Human Brain Development and Interneuron Migration"}]},{"@id":"https://cir.nii.ac.jp/crid/1362825894552136832","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"The\n            <i>C9orf72</i>\n            GGGGCC Repeat Is Translated into Aggregating Dipeptide-Repeat Proteins in FTLD/ALS"}]},{"@id":"https://cir.nii.ac.jp/crid/1362825894626769152","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Amyloid precursor protein (APP) contributes to pathology in the SOD1G93A mouse model of amyotrophic lateral sclerosis"}]},{"@id":"https://cir.nii.ac.jp/crid/1362825894652897664","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Mutations in FUS, an RNA Processing Protein, Cause Familial Amyotrophic Lateral Sclerosis Type 6"}]},{"@id":"https://cir.nii.ac.jp/crid/1362825894836952576","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Missense mutation in the <i>tubulin-specific chaperone E</i> (<i>Tbce</i>) gene in the mouse mutant <i>progressive motor neuronopathy</i>, a model of human motoneuron disease"}]},{"@id":"https://cir.nii.ac.jp/crid/1362825895014012160","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"The “Dying-Back” Phenomenon of Motor Neurons in ALS"}]},{"@id":"https://cir.nii.ac.jp/crid/1362825895038913152","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Neuregulin/ErbB regulate neuromuscular junction development by phosphorylation of α-dystrobrevin"}]},{"@id":"https://cir.nii.ac.jp/crid/1362825895072782976","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Astrocytes expressing ALS-linked mutated SOD1 release factors selectively toxic to motor neurons"}]},{"@id":"https://cir.nii.ac.jp/crid/1362825895137302912","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Fine Structural Observations of Neurofilamentous Changes in Amyotrophic Lateral Sclerosis"}]},{"@id":"https://cir.nii.ac.jp/crid/1362825895954494848","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Insights into the roles of local translation from the axonal transcriptome"}]},{"@id":"https://cir.nii.ac.jp/crid/1362825896228883328","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Onset and Progression in Inherited ALS Determined by Motor Neurons and Microglia"}]},{"@id":"https://cir.nii.ac.jp/crid/1363107368304931456","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Identification of a lectin causing the degeneration of neuronal processes using engineered embryonic stem cells"}]},{"@id":"https://cir.nii.ac.jp/crid/1363107368401684352","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"ALS-Linked SOD1 Mutants Enhance Neurite Outgrowth and Branching in Adult Motor Neurons"}]},{"@id":"https://cir.nii.ac.jp/crid/1363107368451290240","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Messenger RNAs localized to distal projections of human stem cell derived neurons"}]},{"@id":"https://cir.nii.ac.jp/crid/1363107368521172352","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"FUS Phase Separation Is Modulated by a Molecular Chaperone and Methylation of Arginine Cation-π Interactions"}]},{"@id":"https://cir.nii.ac.jp/crid/1363107369257595392","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Cell diversity and network dynamics in photosensitive human brain organoids"}]},{"@id":"https://cir.nii.ac.jp/crid/1363107369645379712","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Rapid Cue-Specific Remodeling of the Nascent Axonal Proteome"}]},{"@id":"https://cir.nii.ac.jp/crid/1363107369687482496","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Impaired mitochondrial dynamics underlie axonal defects in hereditary spastic paraplegias"}]},{"@id":"https://cir.nii.ac.jp/crid/1363107369811574784","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Reduction of Cocaine-Induced Locomotor Effects by Enriched Environment Is Associated with Cell-Specific Accumulation of ΔFosB in Striatal and Cortical Subregions"}]},{"@id":"https://cir.nii.ac.jp/crid/1363107369854934016","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Knockdown of the survival motor neuron (Smn) protein in zebrafish causes defects in motor axon outgrowth and pathfinding"}]},{"@id":"https://cir.nii.ac.jp/crid/1363107369967698176","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Abnormal mitochondrial transport and morphology are common pathological denominators in SOD1 and TDP43 ALS mouse models"}]},{"@id":"https://cir.nii.ac.jp/crid/1363107370037133824","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Axonal Degeneration during Aging and Its Functional Role in Neurodegenerative Disorders"}]},{"@id":"https://cir.nii.ac.jp/crid/1363107370057427072","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Oligodendrocyte dysfunction in the pathogenesis of amyotrophic lateral sclerosis"}]},{"@id":"https://cir.nii.ac.jp/crid/1363107370143902208","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"C9ORF72 interaction with cofilin modulates actin dynamics in motor neurons"}]},{"@id":"https://cir.nii.ac.jp/crid/1363107370327701120","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Mutations in Cu/Zn superoxide dismutase gene are associated with familial amyotrophic lateral sclerosis"}]},{"@id":"https://cir.nii.ac.jp/crid/1363107370568393728","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Intrinsic Membrane Hyperexcitability of Amyotrophic Lateral Sclerosis Patient-Derived Motor Neurons"}]},{"@id":"https://cir.nii.ac.jp/crid/1363107370763854976","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Oligodendroglia: metabolic supporters of axons"}]},{"@id":"https://cir.nii.ac.jp/crid/1363107370927376896","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Altered Reactivity of Superoxide Dismutase in Familial Amyotrophic Lateral Sclerosis"}]},{"@id":"https://cir.nii.ac.jp/crid/1363107370961408896","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Subcellular Profiling Reveals Distinct and Developmentally Regulated Repertoire of Growth Cone mRNAs"}]},{"@id":"https://cir.nii.ac.jp/crid/1363107371094736640","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Neurobiology of axonal transport defects in motor neuron diseases: Opportunities for translational research?"}]},{"@id":"https://cir.nii.ac.jp/crid/1363107371315833472","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"NEK1 variants confer susceptibility to amyotrophic lateral sclerosis"}]},{"@id":"https://cir.nii.ac.jp/crid/1363388843311677440","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"p38α stress-activated protein kinase phosphorylates neurofilaments and is associated with neurofilament pathology in amyotrophic lateral sclerosis"}]},{"@id":"https://cir.nii.ac.jp/crid/1363388843919221248","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Accumulating Evidence for Axonal Translation in Neuronal Homeostasis"}]},{"@id":"https://cir.nii.ac.jp/crid/1363388844229296768","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Regulation of motor proteins, axonal transport deficits and adult-onset neurodegenerative diseases"}]},{"@id":"https://cir.nii.ac.jp/crid/1363388844295306624","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"FUS-mediated regulation of acetylcholine receptor transcription at neuromuscular junctions is compromised in amyotrophic lateral sclerosis"}]},{"@id":"https://cir.nii.ac.jp/crid/1363388844309251968","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Activity-dependent spatially localized miRNA maturation in neuronal dendrites"}]},{"@id":"https://cir.nii.ac.jp/crid/1363388844692257536","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Novel antibodies reveal presynaptic localization of C9orf72 protein and reduced protein levels in C9orf72 mutation carriers"}]},{"@id":"https://cir.nii.ac.jp/crid/1363388844711850368","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Genetic Convergence Brings Clarity to the Enigmatic Red Line in ALS"}]},{"@id":"https://cir.nii.ac.jp/crid/1363388844740781312","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Modeling Environmentally-Induced Motor Neuron Degeneration in Zebrafish"}]},{"@id":"https://cir.nii.ac.jp/crid/1363388844926515840","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Deficiency of the Survival of Motor Neuron Protein Impairs mRNA Localization and Local Translation in the Growth Cone of Motor Neurons"}]},{"@id":"https://cir.nii.ac.jp/crid/1363388845013777536","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Amyotrophic lateral sclerosis-associated mutant SOD1 inhibits anterograde axonal transport of mitochondria by reducing Miro1 levels"}]},{"@id":"https://cir.nii.ac.jp/crid/1363388845029340160","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"ALS Along the Axons – Expression of Coding and Noncoding RNA Differs in Axons of ALS models"}]},{"@id":"https://cir.nii.ac.jp/crid/1363388845093854464","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Focal loss of the glutamate transporter EAAT2 in a transgenic rat model of SOD1 mutant-mediated amyotrophic lateral sclerosis (ALS)"}]},{"@id":"https://cir.nii.ac.jp/crid/1363388845190316160","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Axon-Seq Decodes the Motor Axon Transcriptome and Its Modulation in Response to ALS"}]},{"@id":"https://cir.nii.ac.jp/crid/1363388845699241472","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Inhibition of Axon Regeneration by Liquid-like TIAR-2 Granules"}]},{"@id":"https://cir.nii.ac.jp/crid/1363388845705979520","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Deletion of the BH3-only protein\n            <i>puma</i>\n            protects motoneurons from ER stress-induced apoptosis and delays motoneuron loss in ALS mice"}]},{"@id":"https://cir.nii.ac.jp/crid/1363388845891799680","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Spinal cord endoplasmic reticulum stress associated with a microsomal accumulation of mutant superoxide dismutase-1 in an ALS model"}]},{"@id":"https://cir.nii.ac.jp/crid/1363388846075287040","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Mitochondria Coordinate Sites of Axon Branching through Localized Intra-axonal Protein Synthesis"}]},{"@id":"https://cir.nii.ac.jp/crid/1363670318219161472","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Point mutations of the p150 subunit of\n            <i>dynactin</i>\n            (\n            <i>DCTN1</i>\n            ) gene in ALS"}]},{"@id":"https://cir.nii.ac.jp/crid/1363670318289631232","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Genome-wide identification of mRNAs associated with the protein SMN whose depletion decreases their axonal localization"}]},{"@id":"https://cir.nii.ac.jp/crid/1363670318470378752","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Neurofilaments in the diagnosis of motoneuron diseases: a prospective study on 455 patients"}]},{"@id":"https://cir.nii.ac.jp/crid/1363670318504376192","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Axonal mRNA localization and local protein synthesis in nervous system assembly, maintenance and repair"}]},{"@id":"https://cir.nii.ac.jp/crid/1363670318711737088","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Familial amyotrophic lateral sclerosis-linked SOD1 mutants perturb fast axonal transport to reduce axonal mitochondria content"}]},{"@id":"https://cir.nii.ac.jp/crid/1363670318878728576","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Mitofusin 2 Regulates Axonal Transport of Calpastatin to Prevent Neuromuscular Synaptic Elimination in Skeletal Muscles"}]},{"@id":"https://cir.nii.ac.jp/crid/1363670319604581760","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Exome-wide Rare Variant Analysis Identifies TUBA4A Mutations Associated with Familial ALS"}]},{"@id":"https://cir.nii.ac.jp/crid/1363670319611256320","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"ALS-implicated protein TDP-43 sustains levels of STMN2, a mediator of motor neuron growth and repair"}]},{"@id":"https://cir.nii.ac.jp/crid/1363670319656611712","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"TARDBP mutations in individuals with sporadic and familial amyotrophic lateral sclerosis"}]},{"@id":"https://cir.nii.ac.jp/crid/1363670319669489024","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Regulation of NGF Signaling by an Axonal Untranslated mRNA"}]},{"@id":"https://cir.nii.ac.jp/crid/1363670319849844480","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"iPS cell technologies: significance and applications to CNS regeneration and disease"}]},{"@id":"https://cir.nii.ac.jp/crid/1363670319932421888","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Axonal transcription factors: Novel regulators of growth cone‐to‐nucleus signaling"}]},{"@id":"https://cir.nii.ac.jp/crid/1363670319990422016","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"The human motor neuron axonal transcriptome is enriched for transcripts related to mitochondrial function and microtubule-based axonal transport"}]},{"@id":"https://cir.nii.ac.jp/crid/1363670320133460096","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"The neuron-specific kinesin superfamily protein KIF1A is a uniqye monomeric motor for anterograde axonal transport of synaptic vesicle precursors"}]},{"@id":"https://cir.nii.ac.jp/crid/1363670320492644480","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"SMN regulates axonal local translation via miR-183/mTOR pathway"}]},{"@id":"https://cir.nii.ac.jp/crid/1363670320702299008","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"A<i>De Novo</i><i>RAPGEF2</i>Variant Identified in a Sporadic Amyotrophic Lateral Sclerosis Patient Impairs Microtubule Stability and Axonal Mitochondria Distribution"}]},{"@id":"https://cir.nii.ac.jp/crid/1363670321044283904","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Dysfunction in endoplasmic reticulum-mitochondria crosstalk underlies SIGMAR1 loss of function mediated motor neuron degeneration"}]},{"@id":"https://cir.nii.ac.jp/crid/1363670321301490176","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Structural insights into the interaction and disease mechanism of neurodegenerative disease-associated optineurin and TBK1 proteins"}]},{"@id":"https://cir.nii.ac.jp/crid/1363951793306253312","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Expanded GGGGCC Hexanucleotide Repeat in Noncoding Region of C9ORF72 Causes Chromosome 9p-Linked FTD and ALS"}]},{"@id":"https://cir.nii.ac.jp/crid/1363951793610308352","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Microarray Analysis of the Cellular Pathways Involved in the Adaptation to and Progression of Motor Neuron Injury in the SOD1 G93A Mouse Model of Familial ALS"}]},{"@id":"https://cir.nii.ac.jp/crid/1363951793762932352","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Extracellular stimuli specifically regulate localized levels of individual neuronal mRNAs"}]},{"@id":"https://cir.nii.ac.jp/crid/1363951794314437504","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Late Endosomes Act as mRNA Translation Platforms and Sustain Mitochondria in Axons"}]},{"@id":"https://cir.nii.ac.jp/crid/1363951794591220736","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Toxic gain of function from mutant\n                    <scp>FUS</scp>\n                    protein is crucial to trigger cell autonomous motor neuron loss"}]},{"@id":"https://cir.nii.ac.jp/crid/1363951794792446976","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Phase Separation of FUS Is Suppressed by Its Nuclear Import Receptor and Arginine Methylation"}]},{"@id":"https://cir.nii.ac.jp/crid/1363951794882596480","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Whole transcriptome profiling reveals the RNA content of motor axons"}]},{"@id":"https://cir.nii.ac.jp/crid/1363951794999155968","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"The dendritic spine morphogenic effects of repeated cocaine use occur through the regulation of serum response factor signaling"}]},{"@id":"https://cir.nii.ac.jp/crid/1363951795095266432","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Compartment-Specific Regulation of Autophagy in Primary Neurons"}]},{"@id":"https://cir.nii.ac.jp/crid/1363951795524209280","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Axonal Transport of TDP-43 mRNA Granules Is Impaired by ALS-Causing Mutations"}]},{"@id":"https://cir.nii.ac.jp/crid/1363951795987033728","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Transcriptome analysis of embryonic and adult sensory axons reveals changes in mRNA repertoire localization"}]},{"@id":"https://cir.nii.ac.jp/crid/1363951796171484928","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"ALS as a distal axonopathy: molecular mechanisms affecting neuromuscular junction stability in the presymptomatic stages of the disease"}]},{"@id":"https://cir.nii.ac.jp/crid/1364233268307141888","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"RIPK1 mediates axonal degeneration by promoting inflammation and necroptosis in ALS"}]},{"@id":"https://cir.nii.ac.jp/crid/1364233269152872320","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Mechanisms for the maintenance and regulation of axonal energy supply"}]},{"@id":"https://cir.nii.ac.jp/crid/1364233269551896192","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Subcellular transcriptomes and proteomes of developing axon projections in the cerebral cortex"}]},{"@id":"https://cir.nii.ac.jp/crid/1364233269575618432","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Patient-derived frontotemporal lobar degeneration brain extracts induce formation and spreading of TDP-43 pathology in vivo"}]},{"@id":"https://cir.nii.ac.jp/crid/1364233269735698048","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Oligodendroglia metabolically support axons and contribute to neurodegeneration"}]},{"@id":"https://cir.nii.ac.jp/crid/1364233269940077568","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"ALS‐associated fused in sarcoma (FUS) mutations disrupt Transportin‐mediated nuclear import"}]},{"@id":"https://cir.nii.ac.jp/crid/1364233269986244352","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"The RNA-binding protein SFPQ orchestrates an RNA regulon to promote axon viability"}]},{"@id":"https://cir.nii.ac.jp/crid/1364233270436787200","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Nuclear Import Receptor Inhibits Phase Separation of FUS through Binding to Multiple Sites"}]},{"@id":"https://cir.nii.ac.jp/crid/1364233270586236672","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"CMT2D neuropathy is linked to the neomorphic binding activity of glycyl-tRNA synthetase"}]},{"@id":"https://cir.nii.ac.jp/crid/1364233270625458816","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Axonal damage markers in cerebrospinal fluid are increased in ALS"}]},{"@id":"https://cir.nii.ac.jp/crid/1364233270661690240","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Mutations in the\n                    <i>FUS/TLS</i>\n                    Gene on Chromosome 16 Cause Familial Amyotrophic Lateral Sclerosis"}]},{"@id":"https://cir.nii.ac.jp/crid/1364233270693796224","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"A missense mutation in Tbce causes progressive motor neuronopathy in mice"}]},{"@id":"https://cir.nii.ac.jp/crid/1364233270732516480","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Presynaptic<i>Fmr1</i>Genotype Influences the Degree of Synaptic Connectivity in a Mosaic Mouse Model of Fragile X Syndrome"}]},{"@id":"https://cir.nii.ac.jp/crid/1364233270788061952","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"A novel method to study the local mitochondrial fusion in myelinated axons in vivo"}]},{"@id":"https://cir.nii.ac.jp/crid/1364233270810957696","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Shortened TDP43 isoforms upregulated by neuronal hyperactivity drive TDP43 pathology in ALS"}]},{"@id":"https://cir.nii.ac.jp/crid/1364233271075304960","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Novel Mutations in TARDBP (TDP-43) in Patients with Familial Amyotrophic Lateral Sclerosis"}]},{"@id":"https://cir.nii.ac.jp/crid/1364233271151654016","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Fasudil, a rho kinase inhibitor, limits motor neuron loss in experimental models of amyotrophic lateral sclerosis"}]},{"@id":"https://cir.nii.ac.jp/crid/1390021072389089792","@type":"Article","relationType":["isReferencedBy"],"jpcoar:relatedTitle":[{"@language":"en","@value":"Latest Trends in ALS Drug Development and Integration with Rehabilitation Medicine"},{"@language":"ja","@value":"ALSの治療開発の最新動向とリハビリテーション医療との融合"}]}],"dataSourceIdentifier":[{"@type":"CROSSREF","@value":"10.3389/fnins.2020.00194"},{"@type":"KAKEN","@value":"PRODUCT-22685728"},{"@type":"OPENAIRE","@value":"doi_dedup___::5412a80d34f45772930591f85e384a60"},{"@type":"CROSSREF","@value":"10.2490/jjrmc.61.577_references_DOI_PW9POJbNiRdZg5fp9NHbWno1TQJ"},{"@type":"CROSSREF","@value":"10.1038/s10038-022-01055-8_references_DOI_PW9POJbNiRdZg5fp9NHbWno1TQJ"}]}