3hsh

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Template:STRUCTURE 3hsh

Contents 1 Crystal structure of human collagen XVIII trimerization domain (Tetragonal crystal form) 2 Disease 3 Function 4 About this Structure 5 See Also 6 Reference

Crystal structure of human collagen XVIII trimerization domain (Tetragonal crystal form)

Template:ABSTRACT PUBMED 19631658

Disease

[COIA1_HUMAN] Defects in COL18A1 are a cause of Knobloch syndrome type 1 (KNO1) [MIM:267750]. An autosomal recessive disorder defined by the occurrence of high myopia, vitreoretinal degeneration with retinal detachment, macular abnormalities and occipital encephalocele.^[1]

Function

[COIA1_HUMAN] COLA18A probably plays a major role in determining the retinal structure as well as in the closure of the neural tube. Endostatin potently inhibits endothelial cell proliferation and angiogenesis. May inhibit angiogenesis by binding to the heparan sulfate proteoglycans involved in growth factor signaling.

About this Structure

3hsh is a 6 chain structure with sequence from Homo sapiens. Full crystallographic information is available from OCA.

See Also

• Collagen

Reference

• Boudko SP, Sasaki T, Engel J, Lerch TF, Nix J, Chapman MS, Bachinger HP. Crystal structure of human collagen XVIII trimerization domain: A novel collagen trimerization Fold. J Mol Biol. 2009 Sep 25;392(3):787-802. Epub 2009 Jul 23. PMID:19631658 doi:10.1016/j.jmb.2009.07.057

1. ↑ Sertie AL, Sossi V, Camargo AA, Zatz M, Brahe C, Passos-Bueno MR. Collagen XVIII, containing an endogenous inhibitor of angiogenesis and tumor growth, plays a critical role in the maintenance of retinal structure and in neural tube closure (Knobloch syndrome). Hum Mol Genet. 2000 Aug 12;9(13):2051-8. PMID:10942434