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Here, we examined the role of anthrax toxin receptor 2 (ANTXR2\/CMG2), an ECM turnover receptor whose loss-of-function mutations cause hyaline fibromatosis syndrome (HFS), a multisystem connective tissue disorder. We show that ANTXR2 is expressed predominantly by fibro-adipogenic progenitors (FAPs) and mediates internalization of the major ECM component collagen VI (COL6). ANTXR2-null (Antxr2\u2212\/\u2212) mice exhibit premature COL6 accumulation within intramuscular connective tissue, leading to tissue stiffening and progressive, maladaptive muscle hypertrophy with impaired locomotion and myopathic features. Using Antxr2\u2212\/\u2212::Col6a1\u2212\/\u2212 double-knockout mice, we demonstrate that these phenotypes are COL6-dependent, highlighting the essential role of ANTXR2-mediated COL6 remodeling in preserving ECM homeostasis and skeletal muscle function and establishing a mechanistic basis for muscle pathology in HFS.<\/jats:p>","DOI":"10.1083\/jcb.202606056","type":"journal-article","created":{"date-parts":[[2026,8,26]],"date-time":"2026-08-26T06:21:13Z","timestamp":1787725273000},"update-policy":"https:\/\/doi.org\/10.1083\/jcb.crossmarkpolicy","source":"Crossref","is-referenced-by-count":0,"title":["FAPs orchestrate ANTXR2-mediated fine-tuning of collagen VI to ensure skeletal muscle 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