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  1. 学術雑誌論文
  2. その他
  1. 部局別インデックス
  2. 医学部

Alternative Splicing Regulator RBM20 and Cardiomyopathy

http://hdl.handle.net/20.500.12000/48367
http://hdl.handle.net/20.500.12000/48367
c0b95e7e-f368-42ae-aee0-910fae8b1e0c
名前 / ファイル ライセンス アクション
fmolb-05-00105.pdf fmolb-05-00105.pdf
Item type デフォルトアイテムタイプ(フル)(1)
公開日 2021-04-27
タイトル
タイトル Alternative Splicing Regulator RBM20 and Cardiomyopathy
言語 en
作成者 Watanabe, Takeshi

× Watanabe, Takeshi

en Watanabe, Takeshi

Kimura, Akinori

× Kimura, Akinori

en Kimura, Akinori

Kuroyanagi, Hidehito

× Kuroyanagi, Hidehito

en Kuroyanagi, Hidehito

アクセス権
アクセス権 open access
アクセス権URI http://purl.org/coar/access_right/c_abf2
権利情報
言語 ja
権利情報 © 2018 Watanabe, Kimura and Kuroyanagi.
主題
言語 en
主題Scheme Other
主題 RBM20
言語 en
主題Scheme Other
主題 dilated cardiomyopathy (DCM)
言語 en
主題Scheme Other
主題 alternative splicing
言語 en
主題Scheme Other
主題 isoform switching
言語 en
主題Scheme Other
主題 mutation
言語 en
主題Scheme Other
主題 arginine/serine (RS)-rich region
言語 en
主題Scheme Other
主題 titin
言語 en
主題Scheme Other
主題 nuclear localization
内容記述
内容記述タイプ Other
内容記述 RBM20 is a vertebrate-specific RNA-binding protein with two zinc finger (ZnF) domains, one RNA-recognition motif (RRM)-type RNA-binding domain and an arginine/serine (RS)-rich region. RBM20 has initially been identified as one of dilated cardiomyopathy (DCM)-linked genes. RBM20 is a regulator of heart-specific alternative splicing and Rbm20^<ΔRRM> mice lacking the RRM domain are defective in the splicing regulation. The Rbm20^<ΔRRM> mice, however, do not exhibit a characteristic DCM-like phenotype such as dilatation of left ventricles or systolic dysfunction. Considering that most of the RBM20 mutations identified in familial DCM cases were heterozygous missense mutations in an arginine-serine-arginine-serine-proline (RSRSP) stretch whose phosphorylation is crucial for nuclear localization of RBM20, characterization of a knock-in animal model is awaited. One of the major targets for RBM20 is the TTN gene, which is comprised of the largest number of exons in mammals. Alternative splicing of the TTN gene is exceptionally complicated and RBM20 represses >160 of its consecutive exons, yet detailed mechanisms for such extraordinary regulation are to be elucidated. The TTN gene encodes the largest known protein titin, a multi-functional sarcomeric structural protein specific to striated muscles. As titin is the most important factor for passive tension of cardiomyocytes, extensive heart-specific and developmentally regulated alternative splicing of the TTN pre-mRNA by RBM20 plays a critical role in passive stiffness and diastolic function of the heart. In disease models with diastolic dysfunctions, the phenotypes were rescued by increasing titin compliance through manipulation of the Ttn pre-mRNA splicing, raising RBM20 as a potential therapeutic target.
内容記述タイプ Other
内容記述 論文
出版者
言語 en
出版者 Frontiers Media
言語
言語 eng
資源タイプ
資源タイプ journal article
資源タイプ識別子 http://purl.org/coar/resource_type/c_6501
出版タイプ
出版タイプ VoR
出版タイプResource http://purl.org/coar/version/c_970fb48d4fbd8a85
識別子
識別子 http://hdl.handle.net/20.500.12000/48367
識別子タイプ HDL
関連情報
関連識別子
識別子タイプ DOI
関連識別子 https://doi.org/10.3389/fmolb.2018.00105
関連識別子
識別子タイプ DOI
関連識別子 https://doi.org/10.3389/fmolb.2018.00105
収録物識別子
収録物識別子タイプ ISSN
収録物識別子 2296-889X
収録物名
言語 en
収録物名 Frontiers in Molecular Biosciences
書誌情報
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