Regulation of myonuclear positioning and muscle function by the skeletal muscle-specific CIP protein

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MetadadosDescriçãoIdioma
Autor(es): dc.contributorHarvard Med Sch-
Autor(es): dc.contributorSun Yat Sen Univ-
Autor(es): dc.contributorChongqing Med Univ-
Autor(es): dc.contributorUniversidade de São Paulo (USP)-
Autor(es): dc.contributorSichuan Agr Univ-
Autor(es): dc.contributorUniversidade Estadual Paulista (Unesp)-
Autor(es): dc.contributorNanjing Normal Univ-
Autor(es): dc.contributorHarvard Univ-
Autor(es): dc.contributorVertex Pharmaceut-
Autor(es): dc.creatorLiu, Jianming-
Autor(es): dc.creatorHuang, Zhan-Peng-
Autor(es): dc.creatorNie, Mao-
Autor(es): dc.creatorWang, Gang-
Autor(es): dc.creatorSilva, William J.-
Autor(es): dc.creatorYang, Qiumei-
Autor(es): dc.creatorFreire, Paula P. [UNESP]-
Autor(es): dc.creatorHu, Xiaoyun-
Autor(es): dc.creatorChen, Huaqun-
Autor(es): dc.creatorDeng, Zhongliang-
Autor(es): dc.creatorWilliam, T.-
Autor(es): dc.creatorWang, Da-Zhi-
Data de aceite: dc.date.accessioned2022-02-22T00:11:36Z-
Data de disponibilização: dc.date.available2022-02-22T00:11:36Z-
Data de envio: dc.date.issued2020-12-09-
Data de envio: dc.date.issued2020-12-09-
Data de envio: dc.date.issued2020-08-11-
Fonte completa do material: dc.identifierhttp://dx.doi.org/10.1073/pnas.1922911117-
Fonte completa do material: dc.identifierhttp://hdl.handle.net/11449/197220-
Fonte: dc.identifier.urihttp://educapes.capes.gov.br/handle/11449/197220-
Descrição: dc.descriptionThe appropriate arrangement of myonuclei within skeletal muscle myofibers is of critical importance for normal muscle function, and improper myonuclear localization has been linked to a variety of skeletal muscle diseases, such as centronuclear myopathy and muscular dystrophies. However, the molecules that govern myonuclear positioning remain elusive. Here, we report that skeletal muscle-specific CIP (sk-CIP) is a regulator of nuclear positioning. Genetic deletion of sk-CIP in mice results in misalignment of myonuclei along the myofibers and at specialized structures such as neuromuscular junctions (NMJs) and myotendinous junctions (MT.Is) in vivo, impairing myonuclear positioning after muscle regeneration, leading to severe muscle dystrophy in mdx mice, a mouse model of Duchenne muscular dystrophy. sk-CIP is localized to the centrosome in myoblasts and relocates to the outer nuclear envelope in myotubes upon differentiation. Mechanistically, we found that sk-CIP interacts with the Linker of Nucleoskeleton and Cytoskeleton (LINC) complex and the centriole Microtubule Organizing Center (MTOC) proteins to coordinately modulate myonuclear positioning and alignment. These findings indicate that sk-CIP may function as a muscle-specific anchoring protein to regulate nuclear position in multinucleated muscle cells.-
Descrição: dc.descriptionNIH-
Descrição: dc.descriptionMuscular Dystrophy Association Grant-
Descrição: dc.descriptionMuscular Dystrophy Association Development Grant-
Descrição: dc.descriptionNational Natural Science Foundation of China-
Descrição: dc.descriptionGuangdong Science and Technology Department-
Descrição: dc.descriptionAmerican Heart Association Scientist Development Grant-
Descrição: dc.descriptionHarvard Med Sch, Boston Childrens Hosp, Dept Cardiol, Boston, MA 02115 USA-
Descrição: dc.descriptionSun Yat Sen Univ, Affiliated Hosp 1, Natl Hlth Commiss Key Lab Assisted Circulat, Ctr Translat Med, Guangzhou 510275, Peoples R China-
Descrição: dc.descriptionChongqing Med Univ, Affiliated Hosp 2, Dept Orthopaed Surg, Chongqing 400010, Peoples R China-
Descrição: dc.descriptionUniv Sao Paulo, Lab Biol Celular & Mol Musculo Estriado, BR-05508000 Sao Paulo, Brazil-
Descrição: dc.descriptionSichuan Agr Univ, Dept Anim Sci, Chengdu 611130, Peoples R China-
Descrição: dc.descriptionSao Paulo State Univ, Inst Biosci, Dept Morphol, BR-18618000 Sao Paulo, Brazil-
Descrição: dc.descriptionNanjing Normal Univ, Dept Biol, Nanjing 225300, Peoples R China-
Descrição: dc.descriptionHarvard Univ, Harvard Stem Cell Inst, Cambridge, MA 02138 USA-
Descrição: dc.descriptionVertex Pharmaceut, Vertex Cell & Genet Therapies, Watertown, MA 02472 USA-
Descrição: dc.descriptionSao Paulo State Univ, Inst Biosci, Dept Morphol, BR-18618000 Sao Paulo, Brazil-
Descrição: dc.descriptionNIH: HL116919-
Descrição: dc.descriptionNIH: HL125925-
Descrição: dc.descriptionNIH: T32HL007572-
Descrição: dc.descriptionMuscular Dystrophy Association Grant: 294854-
Descrição: dc.descriptionMuscular Dystrophy Association Development Grant: 186548-
Descrição: dc.descriptionNational Natural Science Foundation of China: 81873463-
Descrição: dc.descriptionGuangdong Science and Technology Department: 2018A050506026-
Descrição: dc.descriptionAmerican Heart Association Scientist Development Grant: 16SDG29760000-
Formato: dc.format19254-19265-
Idioma: dc.languageen-
Publicador: dc.publisherNatl Acad Sciences-
Relação: dc.relationProceedings Of The National Academy Of Sciences Of The United States Of America-
???dc.source???: dc.sourceWeb of Science-
Palavras-chave: dc.subjectskeletal muscle-
Palavras-chave: dc.subjectnuclear positioning-
Palavras-chave: dc.subjectsk-CIP protein-
Palavras-chave: dc.subjectLINC complex-
Palavras-chave: dc.subjectMTOC-
Título: dc.titleRegulation of myonuclear positioning and muscle function by the skeletal muscle-specific CIP protein-
Tipo de arquivo: dc.typelivro digital-
Aparece nas coleções:Repositório Institucional - Unesp

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