FAPs orchestrate ANTXR2-mediated fine-tuning of collagen VI to ensure skeletal muscle function.

Loss of ANTXR2 in mice causes COL6 accumulation, tissue stiffening, and muscle hypertrophy, impairing locomotion and muscle function.

• Why it matters: Disruption of ECM homeostasis is linked to connective tissue disorders like hyaline fibromatosis syndrome, but the mechanisms regulating ECM components such as collagen VI are not fully understood.
• What they did: The study examined ANTXR2 expression in fibro-adipogenic progenitors and used knockout mice to show that ANTXR2 mediates collagen VI internalization, with phenotypes dependent on COL6.
• The result: Findings reveal that ANTXR2-mediated collagen VI remodeling is essential for maintaining ECM balance and skeletal muscle function, providing a mechanistic basis for muscle pathology in HFS.