Collagen VI

Collagen VI (ColVI) is a type of collagen primarily associated with the extracellular matrix of skeletal muscle.[1] ColVI maintains regularity in muscle function and stabilizes the cell membrane.[2] It is synthesized by a complex, multistep pathway that leads to the formation of a unique network of linked microfilaments located in the extracellular matrix (ECM). ColVI plays a vital role in numerous cell types, including chondrocytes, neurons, myocytes, fibroblasts, and cardiomyocytes.[3] ColVI molecules are made up of three alpha chains: α1(VI), α2(VI), and α3(VI).[4] It is encoded by 6 genes: COL6A1, COL6A2, COL6A3, COL6A4, COL6A5, and COL6A6.[3] The chain lengths of α1(VI) and α2(VI) are about 1,000 amino acids. The chain length of α3(VI) is roughly a third larger than those of α1(VI) and α2(VI), and it consists of several spliced variants within the range of 2,500 to 3,100 amino acids.[5]

The first two alpha chains subunits of ColVI have a molecular weight of 140-150 KDa and the third polypeptide chain is larger with a molecular weight of 250-300kDa.[5] ColVI is also found in the skin, lungs, blood vessels, cornea and intervertebral disc. It also forms part of the peripheral nerves, brain, myocardium and adipose tissue.[5]

  1. ^ Merlini L, Angelin A, Tiepolo T, Braghetta P, Sabatelli P, Zamparelli A, Ferlini A, Maraldi NM, Bonaldo P, Bernardi P (April 2008). "Cyclosporin A corrects mitochondrial dysfunction and muscle apoptosis in patients with collagen VI myopathies". Proceedings of the National Academy of Sciences of the United States of America. 105 (13): 5225–9. doi:10.1073/pnas.0800962105. PMC 2278179. PMID 18362356.
  2. ^ Bernardi P, Bonaldo P (December 2008). "Dysfunction of mitochondria and sarcoplasmic reticulum in the pathogenesis of collagen VI muscular dystrophies". Annals of the New York Academy of Sciences. 1147: 303–11. doi:10.1196/annals.1427.009. PMID 19076452. S2CID 45028337.
  3. ^ a b Collagen VI at a glance Matilde Cescon, Francesca Gattazzo, Peiwen Chen, Paolo Bonaldo J Cell Sci 2015 128: 3525-3531; doi: 10.1242/jcs.169748
  4. ^ Pan TC, Zhang RZ, Arita M, Bogdanovich S, Adams SM, Gara SK, Wagener R, Khurana TS, Birk DE, Chu ML (April 2014). "A mouse model for dominant collagen VI disorders: heterozygous deletion of Col6a3 Exon 16". The Journal of Biological Chemistry. 289 (15): 10293–307. doi:10.1074/jbc.M114.549311. PMC 4036154. PMID 24563484.
  5. ^ a b c Chen P, Cescon M, Bonaldo P (July 2013). "Collagen VI in cancer and its biological mechanisms". Trends in Molecular Medicine. 19 (7): 410–7. doi:10.1016/j.molmed.2013.04.001. PMID 23639582.