{"@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/1390017068996838144.json","@type":"Article","productIdentifier":[{"identifier":{"@type":"DOI","@value":"10.14952/seikagaku.2023.950784"}},{"identifier":{"@type":"NDL_BIB_ID","@value":"033256043"}},{"identifier":{"@type":"URI","@value":"http://id.ndl.go.jp/bib/033256043"}},{"identifier":{"@type":"URI","@value":"https://ndlsearch.ndl.go.jp/books/R000000004-I033256043"}}],"dc:title":[{"@language":"en","@value":"Intracellular crystallizable human neuraminidase 1 and cure of neuraminidase 1 deficiency"},{"@language":"ja","@value":"細胞内結晶化能を持つノイラミニダーゼ1と欠損症の治療法開発"},{"@language":"ja-Kana","@value":"サイボウ ナイ ケッショウカノウ オ モツ ノイラミニダーゼ 1 ト ケッソンショウ ノ チリョウホウ カイハツ"}],"dc:language":"ja","description":[{"type":"abstract","notation":[{"@language":"ja","@value":"タンパク質の結晶化は，一般に高純度に精製したものを試験管内で行うのが一般的である．しかしごく一部のタンパク質は特定の条件を満たすと，細胞内という他のタンパク質が大量に存在する環境で結晶化することが知られている．その中でも，リソソーム性グリコシダーゼの一種であるヒトノイラミニダーゼ1（NEU1）は，ヒトのほぼ全細胞に存在するハウスキーピングタンパク質でありながら，過剰発現させた場合，ヒトの細胞内，特に小胞体内腔で結晶化する．本稿では，NEU1を一つの例として中心的に取り上げ，他の細胞内結晶化を起こすタンパク質，そして細胞内結晶と細胞小器官（オルガネラ）との関係，さらには細胞内結晶化の意義について紹介する．また，NEU1が関連する疾患やその治療法の展望についても概説する．"}],"abstractLicenseFlag":"disallow"}],"creator":[{"@id":"https://cir.nii.ac.jp/crid/1410017068996838145","@type":"Researcher","foaf:name":[{"@language":"ja","@value":"月本 準"},{"@language":"en","@value":"Tsukimoto Jun"}],"jpcoar:affiliationName":[{"@language":"en","@value":"Graduate School of Biomedical Sciences, Pharmaceutical Sciences, Tokushima University"},{"@language":"ja","@value":"徳島大学大学院医歯薬学研究部"}]},{"@id":"https://cir.nii.ac.jp/crid/1410017068996838144","@type":"Researcher","personIdentifier":[{"@type":"ORCID","@value":"0000-0002-2953-5530"}],"foaf:name":[{"@language":"en","@value":"Itoh Kohji"},{"@language":"ja","@value":"伊藤 孝司"}],"jpcoar:affiliationName":[{"@language":"ja","@value":"徳島大学大学院医歯薬学研究部"},{"@language":"en","@value":"Graduate School of Biomedical Sciences, Pharmaceutical Sciences, Tokushima University"},{"@language":"en","@value":"School of Medicine, Jichi Medical University"},{"@language":"ja","@value":"自治医科大学医学部小児科学"}]}],"publication":{"publicationIdentifier":[{"@type":"PISSN","@value":"00371017"},{"@type":"NDL_BIB_ID","@value":"000000012801"},{"@type":"ISSN","@value":"00371017"},{"@type":"LISSN","@value":"00371017"},{"@type":"NCID","@value":"AN00126072"}],"prism:publicationName":[{"@language":"ja","@value":"生化学"}],"dc:publisher":[{"@language":"ja","@value":"公益社団法人日本生化学会"}],"prism:publicationDate":"2023-12-25","prism:volume":"95","prism:number":"6","prism:startingPage":"784","prism:endingPage":"791"},"url":[{"@id":"http://id.ndl.go.jp/bib/033256043"},{"@id":"https://ndlsearch.ndl.go.jp/books/R000000004-I033256043"}],"availableAt":"2023-12-25","dataSourceIdentifier":[{"@type":"JALC","@value":"oai:japanlinkcenter.org:2012254600"},{"@type":"NDL_SEARCH","@value":"oai:ndlsearch.ndl.go.jp:R000000004-I033256043"}]}