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遗传性大疱性表皮松解症。临床特征、分子遗传学及发病机制。

Inherited epidermolysis bullosa. Clinical features, molecular genetics, and pathoetiologic mechanisms.

作者信息

Uitto J, Christiano A M

机构信息

Department of Dermatology, Jefferson Medical College, Thomas Jefferson University, Philadelphia, Pennsylvania.

出版信息

Dermatol Clin. 1993 Jul;11(3):549-63.

PMID:8365039
Abstract

Recent advances in the molecular biology of the extracellular matrix have resulted in a detailed understanding of the cutaneous basement membrane zone. Consequently, the application of this information to the study of the molecular genetics of the genodermatoses has resulted in spectacular success in the last few years. This article summarizes progress made in the molecular biology of the cutaneous basement membrane zone and its impact on understanding the basis of heritable blistering diseases of the skin, with particular emphasis on epidermolysis bullosa.

摘要

细胞外基质分子生物学的最新进展使人们对皮肤基底膜带(cutaneous basement membrane zone)有了详细的了解。因此,在过去几年里,将这些信息应用于遗传性皮肤病(genodermatoses)分子遗传学的研究取得了显著成功。本文总结了皮肤基底膜带分子生物学方面取得的进展及其对理解遗传性皮肤水疱病发病基础的影响,尤其着重于大疱性表皮松解症(epidermolysis bullosa)。

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