基底膜
IV型胶原
胶原蛋白,I型,α1
阿尔波特综合征
细胞生物学
结缔组织
化学
蛋白酵素
Ⅰ型胶原
细胞外基质
层粘连蛋白
病理
生物
生物化学
肾
内分泌学
医学
肾小球肾炎
酶
作者
Jannie Marie Bülow Sand,S.F. Madsen,M.A. Karsdal
出处
期刊:Elsevier eBooks
[Elsevier]
日期:2024-01-01
卷期号:: 37-53
被引量:2
标识
DOI:10.1016/b978-0-443-15617-5.00017-2
摘要
Type IV collagen is the main collagen component of the basement membrane. It is a network-forming collagen that underlies epithelial and endothelial cells and functions as a barrier between tissue compartments. Type IV collagen has many binding partners and is the backbone of the basement membrane. It holds important signaling potential as subdomains, such as tumstatin, and are released when the protein is degraded by specific proteases. Consequently, type IV collagen is the most important structural collagen of the basement membrane, and its key signaling potential makes it important for a variety of physiological and pathological functions. One of the most well-studied mutations in type IV collagen causes Alport syndrome, a chronic kidney disease. Several biomarkers of type IV collagen have been developed, including domains such as 7S and degradation fragments, documenting the importance of type IV collagen turnover in most, if not all, connective tissue diseases.
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