{"@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/1362262943545751936.json","@type":"Article","productIdentifier":[{"identifier":{"@type":"DOI","@value":"10.1093/oxfordjournals.jbchem.a002950"}},{"identifier":{"@type":"URI","@value":"http://academic.oup.com/jb/article-pdf/130/1/127/2461791/130-1-127.pdf"}},{"identifier":{"@type":"PMID","@value":"11432788"}}],"dc:title":[{"@value":"Effects of Troponin T Mutations in Familial Hypertrophic Cardiomyopathy on Regulatory Functions of Other Troponin Subunits"}],"description":[{"notation":[{"@value":"We have previously shown that mutations in troponin T (TnT), which is associated with familial hypertrophic cardiomyopathy (HCM), cause an increase in the Ca(2+) sensitivity and a potentiation of cardiac muscle contraction. To gain further insight into the patho-physiological role of these mutations, four mutations (Arg92Gln, Phe110Ile, Glu244Asp, Arg278Cys) were introduced into recombinant human cardiac TnT, and the mutants were exchanged into isolated porcine cardiac myofibrils. The effects of mutations were tested on maximal ATPase activity, the inhibitory function of troponin I (TnI) in the absence of troponin C (TnC), and the neutralizing function of TnC. Arg92Gln, Phe110Ile, and Glu244Asp markedly impaired the inhibitory function of TnI. Arg278Cys also impaired the inhibitory function of TnI, but the effect was much smaller. Phe110Ile and Glu244Asp markedly enhanced the neutralizing function of TnC and potentiated the maximum ATPase activity. Arg92Gln and Arg278Cys only slightly enhanced the neutralizing function of TnC, and they conferred no potentiation on the maximum ATPase activity. These results indicate that mutations in TnT impair multiple processes of Ca(2+) regulation by troponin, and there are marked differences in the degree of impairment from mutation to mutation."}]}],"creator":[{"@id":"https://cir.nii.ac.jp/crid/1380569646352049155","@type":"Researcher","foaf:name":[{"@value":"F. Takahashi-Yanaga"}]},{"@id":"https://cir.nii.ac.jp/crid/1382262943545751937","@type":"Researcher","foaf:name":[{"@value":"I. Ohtsuki"}]},{"@id":"https://cir.nii.ac.jp/crid/1382262943545751936","@type":"Researcher","foaf:name":[{"@value":"S. Morimoto"}]}],"publication":{"publicationIdentifier":[{"@type":"PISSN","@value":"0021924X"},{"@type":"PISSN","@value":"http://id.crossref.org/issn/0021924X"}],"prism:publicationName":[{"@value":"Journal of Biochemistry"}],"dc:publisher":[{"@value":"Oxford University Press (OUP)"}],"prism:publicationDate":"2001-07-01","prism:volume":"130","prism:number":"1","prism:startingPage":"127","prism:endingPage":"131"},"reviewed":"false","url":[{"@id":"http://academic.oup.com/jb/article-pdf/130/1/127/2461791/130-1-127.pdf"}],"createdAt":"2012-01-21","modifiedAt":"2017-08-23","foaf:topic":[{"@id":"https://cir.nii.ac.jp/all?q=Adenosine%20Triphosphatases","dc:title":"Adenosine Triphosphatases"},{"@id":"https://cir.nii.ac.jp/all?q=Swine","dc:title":"Swine"},{"@id":"https://cir.nii.ac.jp/all?q=Myocardium","dc:title":"Myocardium"},{"@id":"https://cir.nii.ac.jp/all?q=Troponin%20I","dc:title":"Troponin I"},{"@id":"https://cir.nii.ac.jp/all?q=Cardiomyopathy,%20Hypertrophic","dc:title":"Cardiomyopathy, Hypertrophic"},{"@id":"https://cir.nii.ac.jp/all?q=Recombinant%20Proteins","dc:title":"Recombinant Proteins"},{"@id":"https://cir.nii.ac.jp/all?q=Troponin","dc:title":"Troponin"},{"@id":"https://cir.nii.ac.jp/all?q=Protein%20Subunits","dc:title":"Protein Subunits"},{"@id":"https://cir.nii.ac.jp/all?q=Myofibrils","dc:title":"Myofibrils"},{"@id":"https://cir.nii.ac.jp/all?q=Troponin%20T","dc:title":"Troponin T"},{"@id":"https://cir.nii.ac.jp/all?q=Mutation","dc:title":"Mutation"},{"@id":"https://cir.nii.ac.jp/all?q=Animals","dc:title":"Animals"},{"@id":"https://cir.nii.ac.jp/all?q=Humans","dc:title":"Humans"},{"@id":"https://cir.nii.ac.jp/all?q=Troponin%20C","dc:title":"Troponin C"}],"relatedProduct":[{"@id":"https://cir.nii.ac.jp/crid/1390282679249293568","@type":"Article","resourceType":"学術雑誌論文(journal article)","relationType":["isReferencedBy"],"jpcoar:relatedTitle":[{"@language":"en","@value":"Towards Understanding Mechanisms of Drug Action and Functions of the Body on the Molecular Level. Molecular mechanisms of calcium regulation of striated muscle contraction and its genetic disorder."},{"@language":"ja","@value":"薬の作用機序と生体機能の分子的理解に向けて　　筋収縮カルシウム受容調節の分子機構と遺伝性機能障害"},{"@value":"筋収縮カルシウム受容調節の分子機構と遺伝性機能障害"},{"@language":"ja-Kana","@value":"キンシュウシュク カルシウム ジュヨウ チョウセツ ノ ブンシ キコウ ト イデンセイ キノウ ショウガイ"}]}],"dataSourceIdentifier":[{"@type":"CROSSREF","@value":"10.1093/oxfordjournals.jbchem.a002950"},{"@type":"OPENAIRE","@value":"doi_dedup___::b65851b684c3994e85ec22ec42aaed4d"},{"@type":"CROSSREF","@value":"10.1254/fpj.118.147_references_DOI_18vZMTmcE8XwFNuQzgXTNdTSZQA"}]}