Collagen XVII

被引:28
作者
Has, Cristina [1 ]
Kern, Johannes S. [1 ]
机构
[1] Univ Med Ctr Freiburg, Dept Dermatol, D-79104 Freiburg, Germany
关键词
Collagen XVII; Epidermolysis bullosa; Bullous pemphigoid; Dermal-epidermal junction; Epidermal adhesion; JUNCTIONAL EPIDERMOLYSIS-BULLOSA; GLYCINE SUBSTITUTION; REVERTANT MOSAICISM; PEMPHIGOID ANTIGEN; COL17A1; MUTATIONS; MESSENGER-RNA; MILD FORM; ECTODOMAIN; DOMAIN; DIAGNOSIS;
D O I
10.1016/j.det.2009.10.007
中图分类号
R75 [皮肤病学与性病学];
学科分类号
100206 ;
摘要
Collagen XVII has been identified as having a role in inherited junctional epidermolysis bullosa non-Herlitz (JEB-other, MIM #226650). The role of collagen XVII in both autoimmune and genetic blistering disorders demonstrates its relevance to dermal-epidermal adhesion. Collagen XVII is a major structural component of the hemidesmosome (HD), a highly specialized multiprotein complex that mediates the anchorage of basal epithelial cells to the underlying basement membrane in stratified, pseudostratified, and transitional epithelia. This article examines the genetic and pathological features of collagen XVII.
引用
收藏
页码:61 / +
页数:7
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