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Here, we found that skeletal-muscle-specific ablation of the HspB7 does not affect myogenesis during embryonic stages to postnatal day 1 (P1), but causes subsequent postnatal death owing to a respiration defect, with progressive myopathy phenotypes in the diaphragm. Deficiency of HSPB7 in the diaphragm muscle resulted in muscle fibrosis, sarcomere disarray and sarcolemma integrity loss. We identified dimerized filamin C (FLNC) as an interacting partner of HSPB7. Immunofluorescence studies demonstrated that the aggregation and mislocalization of FLNC occurred in the muscle of HspB7 mutant adult mice. Furthermore, the components of dystrophin glycoprotein complex, \u03b3- and \u03b4-sarcoglycan, but not dystrophin, were abnormally upregulated and mislocalized in HSPB7 mutant muscle. Collectively, our findings suggest that HSPB7 is essential for maintaining muscle integrity, which is achieved through its interaction with FLNC, in order to prevent the occurrence and progression of myopathy.<\/jats:p>","DOI":"10.1242\/jcs.179887","type":"journal-article","created":{"date-parts":[[2016,3,1]],"date-time":"2016-03-01T02:12:57Z","timestamp":1456798377000},"page":"1661-1670","source":"Crossref","is-referenced-by-count":58,"title":["HSPB7 interacts with dimerized FLNC and its absence results in progressive myopathy in skeletal muscles"],"prefix":"10.1242","volume":"129","author":[{"given":"Liang-Yi","family":"Juo","sequence":"first","affiliation":[{"name":"Institute of Biochemistry and Molecular Biology, National Yang-Ming University 1 , Taipei 112 , Taiwan"},{"name":"Institute of Biomedical Sciences, Academia Sinica 2 , Taipei 115 , Taiwan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Wern-Chir","family":"Liao","sequence":"additional","affiliation":[{"name":"Institute of Biochemistry and Molecular Biology, National Yang-Ming University 1 , Taipei 112 , Taiwan"},{"name":"Institute of Biomedical Sciences, Academia Sinica 2 , Taipei 115 , Taiwan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yen-Ling","family":"Shih","sequence":"additional","affiliation":[{"name":"Institute of Biomedical Sciences, Academia Sinica 2 , Taipei 115 , Taiwan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Bih-Ying","family":"Yang","sequence":"additional","affiliation":[{"name":"Institute of Biomedical Sciences, Academia Sinica 2 , Taipei 115 , Taiwan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"An-Bang","family":"Liu","sequence":"additional","affiliation":[{"name":"Buddhist Tzu Chi General Hospital and Buddhist Tzu Chi University 3 Department of Neurology , , Hualien 970 , Taiwan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9559-706X","authenticated-orcid":false,"given":"Yu-Ting","family":"Yan","sequence":"additional","affiliation":[{"name":"Institute of Biochemistry and Molecular Biology, National Yang-Ming University 1 , Taipei 112 , Taiwan"},{"name":"Institute of Biomedical Sciences, Academia Sinica 2 , Taipei 115 , Taiwan"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"237","reference":[{"key":"2024061316080423100_JCS179887C1","doi-asserted-by":"publisher","first-page":"143","DOI":"10.1016\/j.cub.2009.11.022","article-title":"Chaperone-assisted selective autophagy is essential for muscle maintenance","volume":"20","author":"Arndt","year":"2010","journal-title":"Curr. 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