基底膜
基因亚型
IV型胶原
胶原蛋白,I型,α1
化学
细胞生物学
错义突变
内皮抑素
血管生成
分子生物学
生物
生物化学
层粘连蛋白
细胞外基质
遗传学
突变
基因
作者
M. Pehrsson,Cecilie Liv Bager,M.A. Karsdal
标识
DOI:10.1016/b978-0-443-15617-5.00021-4
摘要
Type XVIII collagen possesses features of collagens and proteoglycans and is localized in various basement membrane zones. Endostatin, the carboxyl-terminal fragment of type XVIII collagen, is capable of inhibiting angiogenesis and tumor growth. Type XVIII collagen has three isoforms: short, medium, and long. The short isoform is present in vascular and epithelial basement membrane structures, and the long isoform is highly expressed in the liver. Nonsense and missense mutations of type XVIII collagen lead to the autosomal recessive disorder Knobloch syndrome-1, characterized by eye abnormalities. COL18A1−/−-knockout mice suffer from eye abnormalities and manifest broadened basement membrane structures. This suggests that type XVIII collagen is involved in eye development and essential to maintaining basement membrane integrity. Type XVIII collagen is the prime example of collagen, which holds structural properties in its intact form and signaling potential in its degraded form.
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