Collagen XVII
Collagen XVII
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DOI：
10.1016/j.det.2009.10.007
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发表时间：
2010-01-01
影响因子：
2.4
通讯作者：
Kern, Johannes S.
中科院分区：
文献类型：
--
作者：
Has, Cristina;Kern, Johannes S.
胶原蛋白XVII已被鉴定为在遗传性非Herlitz型交界性大疱性表皮病中起作用（JEB-其他，MIM #226650）。胶原蛋白XVII在自身免疫性和遗传性起泡性疾病中的作用表明其与真皮-表皮粘附相关。胶原XVII是半桥粒（HD）的主要结构组分，半桥粒是高度特化的多蛋白复合物，其介导基底上皮细胞在复层、假复层和移行上皮中锚定到下面的基底膜。本文探讨了胶原蛋白XVII的遗传和病理特征。
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.