collagen alpha-1(VIII) chain; endothelial collagen; contains: vastatin (COL8A1, C3orf7)
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Function
- macromolecular component of the subendothelium
- major component of the Descemet's membrane (basement membrane corneal endothelium)
- also component of the endothelium of blood vessels
- necessary for migration & proliferation of vascular smooth muscle cells
- Pro at the third position of the tripeptide repeating unit (G-X-Y) are hydroxylated in some or all of the chains
- proteolytically cleaved by neutrophil elastase, in vitro
- proteolytic processing produces the C-terminal NC1 domain fragment, vastatin
- homotrimers, or heterotrimers in association with COL8A2
- four homotrimers can form a tetrhedron stabilized by central interacting C-terminal NC1 trimers
Structure
- contains 1 C1q domain
- four consecutive Gly-Pro-Pro triplets are present at the C-terminus of the triple-helical region
- these may provide the high thermal stability of this region
Compartment
- secreted, extracellular space
- extracellularmatrix, basement membrane
Expression
- expressed primarily in subendothelium of large blood vessels
- also expressed in arterioles & venules in muscle, heart, kidney, spleen, umbilical cord, liver & lung
- also found in connective tissue layers around hair follicles, around nerve bundles in muscle, in the dura of the optic nerve, in cornea & sclera, & in the perichondrium of cartilage
- tn the kidney, expressed in mesangial cells, glomerular endothelial cells, & tubular epithelial cells
- also expressed in mast cells, & in astrocytes during the repair process
- expressed in Descemet's membrane
- specifically expressed in peritoneal fibroblasts & mesothelial cells
Pathology
- possible role in atherogenesis via effect on proliferation of vascular smooth muscle cells
- up-regulated during vascular injury, in atherosclerosis & in diabetes
More general terms
More specific terms
Component of
References
- ↑ UniProt http://www.uniprot.org/uniprot/P27658.html
- ↑ Molecular Cell Biology (2nd ed) Darnell J; Lodish H & Baltimore D (eds), Scientific American Books, WH Freeman, NY 1990, pg 906
- ↑ Entrez Gene http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&cmd=Retrieve&dopt=Graphics&list_uids=1295