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营养不良型大疱性表皮松解症某些研究现状 被引量:1

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摘要 营养不良型大疱性表皮松解症(dystrophicepidermolysisbullosaDEB)是常染色体遗传性皮肤病,以皮肤黏膜脆性增加,容易出现水疱和大疱,愈后留有萎缩性瘢痕为特征。根据遗传方式又可分为显性遗传(DDEB)和隐性遗传(RDEB),两者都是由编码致密下层锚原纤维的主要成分—VII型胶原基因COL7A1发生突变,导致锚原纤维的数量和质量异常所致。随着分子生物学技术的发展,对DEB发病机制的认识不断深入,新的突变基因不断发现,本文就其目前研究现状作一综述。
出处 《中国麻风皮肤病杂志》 2006年第5期399-402,共4页 China Journal of Leprosy and Skin Diseases
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