Collagen Type VI Regulates TGF-β Bioavailability in Skeletal Muscle in Mice

Collagen Type VI Regulates TGF-β Bioavailability in Skeletal Muscle in Mice

Researchers identified a conspicuous dysregulation of the TGF-β pathway early in the disease process and proposed that the collagen VI–deficient matrix is not capable of regulating the dynamic TGF-β bioavailability both at baseline and in response to muscle injury. [Journal of Clinical Investigation] Full Article

JCI Collagen type VI regulates TGF-β bioavailability in skeletal muscle in mice

Collagen VI–related disorders (COL6-RDs) are a group of rare muscular dystrophies caused by pathogenic variants in collagen VI genes (COL6A1, COL6A2, and COL6A3). 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 mislocalization of collagen VI in the ECM underlies the noncell-autonomous dysf…