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collagen alpha-1(VIII) chain; endothelial collagen; contains: vastatin (COL8A1, C3orf7)
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
Specific
vastatin
General
collagen subunit
Properties
SIZE: entity length = 744 aa
MW = 73 kD
MOTIF: signal sequence {1-27}
Nonhelical region (NC2) {29-117}
Triple-helical region (COL1) {118-571}
Nonhelical region (NC1) {572-744}
MOTIF: C1q {611-744}
Database Correlations
OMIM 120251
UniProt P27658
PFAM correlations
Entrez Gene 1295
Kegg hsa:1295
References
- UniProt :accession P27658
- Molecular Cell Biology (2nd ed) Darnell J; Lodish H
& Baltimore D (eds), Scientific American Books,
WH Freeman, NY 1990, pg 906
- Entrez Gene :accession 1295
Component-of
collagen type-8 (endothelial collagen)