Collagen type VI regulates TGFβ bioavailability in skeletal muscle.

Collagen VI-related disorders (-RDs) are a group of rare muscular dystrophies caused by pathogenic variants in collagen VI genes (, and ). Collagen type VI is a heterotrimeric, microfibrillar component of the muscle extracellular matrix (ECM), predominantly secreted by resident fibroadipogenic precursor cells in skeletal muscle. The absence or mislocalizatoion of collagen VI in the ECM underlies the non-cell autonomous dysfunction and dystrophic changes in skeletal muscle with an as of yet elusive direct mechanistic link between the ECM and myofiber dysfunction.