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Molecular biology and pathology of type VII collagen*

 

作者: Jouni Uitto,   Linda C. Chung‐Honet,   Angela M. Christiano,  

 

期刊: Experimental Dermatology  (WILEY Available online 1992)
卷期: Volume 1, issue 1  

页码: 2-11

 

ISSN:0906-6705

 

年代: 1992

 

DOI:10.1111/j.1600-0625.1992.tb00065.x

 

出版商: Blackwell Publishing Ltd

 

关键词: type VII collagen;anchoring fibrils;epidermolysis bullosa

 

数据来源: WILEY

 

摘要:

AbstractType VII collagen is a genetically distinct member of the collagen family of proteins. Type VII collagen has been shown to be the major component of anchoring fibrils, attachment complexes which secure the cutaneous basement membrance of the skin to the underlying dermis. Understanding of the structure of type VII collagen has been advanced by recent cloning of the corresponding gene. Chromosomal mapping of the gene to the short arm of chromosome 3 and identification of intragenic polymorphic markers have allowed demonstration of strong genetic linkage between the type VII collagen locus and the dystrophic forms of EB (epidermolysis bullosa). This overview summarizes the progress made in the molecular genetics of type VII collagen.

 

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