A member of von Willebrand factor A involved in the formation of filamentous network within extracellular matrix and as endogenous neuronal molecule regulated in association with inflammatory axonal injury.
Matrilin-2 gene is mapped onchromosome 8q22 with 19 exons that contains 956 amino acids, 2 VWFA-like domains, 10EGF-type modules and oligomerization module where mostly expressed in fibroblastic or epithelial origin with two identified transcript variants isoforms differs from each other in a short region.
Matrilin-2 induced proinflammatory responses and reveals to express in lesion but not in normal appearing white matter as such it indicates as a deleterious endogenous neuro-axonal injury response signal that stimulates innate immune cells and contributes to premature axonal damage in central nervous system inflammatory diseases like multiple sclerosis.
Matrilin-2 is one of the members of matrilin family that acts as an oligomeric extracellular adaptor protein wherein outside the central nervous system it is a structural component of the extracellular matrix in varioustissues including skin that linked to various inflammation-associated diseases like cancers and fibrosis. Moreover in peripheral nerve crush injury it has a potential role in peripheral nervous system repair.
Gene name: MATN2
Protein name: Matrilin-2