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collagen 4 alpha-2; contains: canstatin (COL4A2)

Function: - type 4 collagen is the major structural component of glomerular basement membranes (GBM), forming a 'chicken-wire' meshwork together with laminins, proteoglycans & entactin/nidogen - proteolytic processing produces the C-terminal NC1 peptide, canstatin: - has both anti-angiogenic & anti-tumor cell activity - inhibits proliferation & migration of endothelial cells, - reduces mitochondrial membrane potential - induces apoptosis - specifically induces Fas-dependent apoptosis - activates procaspase-8 & procaspase-9 activity - ligand for integrin alpha-5/beta-3 & integrin alpha-5/beta-5 - Pro at the third position of the tripeptide repeating unit (G-X-Y) are hydroxylated in some or all of the chains - type 4 collagen contains numerous Cys which are involved in intermolecular & intramolecular disulfide bonding - 12 of these, located in the NC1 domain, are conserved in all known type 4 collagen - the trimeric structure of the NC1 domains is stabilized by covalent bonds between Lys & Met residues (putative) - there are six type 4 collagen isoforms, each of which can form a triple helix structure with 2 other chains to generate type 4 collagen network Structure: - alpha chains of type 4 collagen have - a non-collagenous domain (NC1) at their C-terminus - frequent interruptions of the G-X-Y repeats in the long central triple-helical domain (which may cause flexibility in the triple helix) - short N-terminal triple-helical 7S domain - belongs to the type 4 collagen family - contains 1 collagen 4 NC1 (C-terminal non-collagenous) domain Compartment: - secreted, extracellular space - extracellular matrix, basement membrane

Specific

canstatin

General

collagen subunit

Properties

SIZE: entity length = 1712 aa MW = 168 kD MOTIF: signal sequence {1-25} N-glycosylation site {N138} Triple-helical region {184-1484} Collagen IV NC1 {1489-1712} MOTIF: cysteine residue {C1504} MODIFICATION: cysteine residue {C1593} cysteine residue {C1537} MODIFICATION: cysteine residue {C1590} cysteine residue {C1549} MODIFICATION: cysteine residue {C1555} cysteine residue {C1555} MODIFICATION: cysteine residue {C1549} cysteine residue {C1590} MODIFICATION: cysteine residue {C1537} cysteine residue {C1593} MODIFICATION: cysteine residue {C1504} cysteine residue {C1612} MODIFICATION: cysteine residue {C1708} cysteine residue {C1646} MODIFICATION: cysteine residue {C1705} cysteine residue {C1658} MODIFICATION: cysteine residue {C1665} cysteine residue {C1665} MODIFICATION: cysteine residue {C1658} cysteine residue {C1705} MODIFICATION: cysteine residue {C1646} cysteine residue {C1708} MODIFICATION: cysteine residue {C1612}

Database Correlations

OMIM 120090 UniProt P08572 PFAM correlations Entrez Gene 1284 Kegg hsa:1284 ENZYME

References

  1. UniProt :accession P08572
  2. Molecular Cell Biology (2nd ed) Darnell J; Lodish H & Baltimore D (eds), Scientific American Books, WH Freeman, NY 1990, pg 906
  3. OMIM :accession 120090
  4. Entrez Gene :accession 1284

Component-of

collagen type-4