The primary two alpha chains subunits of ColVI have a molecular weight of 140-150 KDa and the third polypeptide chain is bigger with a molecular weight of 250-300kDa.[5] ColVI can also be discovered within the pores and skin, lungs, blood vessels, cornea and intervertebral disc. It additionally kinds a part of the peripheral nerves, mind, myocardium and adipose tissue.[5]
Contents
Perform[edit]
ColVI maintains a mechanical perform within the cell, which is typical of most varieties of Collagen, by offering stability and structural help within the ECM. ColVI permits muscle cells to attach with the ECM by interacting with perlecan within the basal lamina.[6] ColVI additionally features as a cytoprotective agent:[3]
Collagen VI performs many various roles within the cell relying on which tissue during which it’s expressed.
Expression in Numerous Tissues[edit]
Research have revealed that the mutation or deletion of genes encoding for collagen VI can lead to quite a few musculoskeletal problems, e.g. hip osteoarthritis, tissue fibrosis, tissue ossification, and muscular dystrophies. The deletion of the COL6A1 gene in mice was used to find out the perform of collagen VI within the bone and cartilage of knee joints. The absence of collagen VI impacted the construction and form of the knee joint, however didn’t critically have an effect on physicality of the cartilage.[24]
Related Problems[edit]
Bethlem myopathy is the mildest type of Collagen VI associated myopathies. Associated signs embody ligamentous laxity, hypotonia in infancy, and problem respiration as a result of weak point in respiratory muscle groups. Bethlem myopathy impacts roughly 1 in 200,000 individuals.[30]