Article

Rewiring Fibroblast–Muscle Axis Drives Progressive Pathology in Bethlem Myopathy

2026-05-18

Abstract excerpt

<h4>ABSTRACT</h4> Collagen VI–related myopathies, including Bethlem myopathy (BM), are progressive muscle disorders, but the mechanisms driving age-dependent disease progression remain poorly understood. Here, we used a zebrafish BM model carrying an exon-skipping mutation that generates a shorter collagen VI α1 chain and disrupts supramolecular assembly, recapitulating key features of the human disease. We furth...

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Literature Corpus work: 7b1b3112-7389-5897-a5b0-75a4a0a99dba
DOI: 10.64898/2026.05.14.725126

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