Most cases are caused by mutations in the genes encoding for the subunits composing type V collagen, an essential component of the extracellular matrix in many of the connective tissues in the body.

The extensive extracellular matrix (ECM) of connective tissues is a complex interacting network of proteins, glycoproteins and proteoglycans providing the dynamic and essential three-dimensional ...

Matrix elements and fibrotic changes. Major components of the extracellular matrix (connective tissue) of the liver and the four most important changes in the fibrotic matrix. Formal pathogenesis ...