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LAMA3基因的部分缺失是美国鞍马遗传性交界性大疱性表皮松解症的病因。

Partial deletion of the LAMA3 gene is responsible for hereditary junctional epidermolysis bullosa in the American Saddlebred Horse.

作者信息

Graves K T, Henney P J, Ennis R B

机构信息

Department of Veterinary Science, University of Kentucky, Lexington, KY 40546, USA.

出版信息

Anim Genet. 2009 Feb;40(1):35-41. doi: 10.1111/j.1365-2052.2008.01795.x. Epub 2008 Nov 11.

Abstract

Laminin 5 is a heterotrimeric basement membrane protein integral to the structure and function of the dermal-epidermal junction. It consists of three glycoprotein subunits: the alpha3, beta3 and gamma2 chains, which are encoded by the LAMA3, LAMB3 and LAMC2 genes respectively. A mutation in any of these genes results in the condition known as hereditary junctional epidermolysis bullosa (JEB). A 6589-bp deletion spanning exons 24-27 was found in the LAMA3 gene in American Saddlebred foals born with the skin-blistering condition epitheliogenesis imperfecta. The deletion confirms that this autosomal recessive condition in the American Saddlebred Horse can indeed be classified as JEB and corresponds to Herlitz JEB in humans. A diagnostic test was developed and nine of 175 randomly selected American Saddlebred foals from the 2007 foal crop were found to be carriers of the mutation (frequency of 0.026).

摘要

层粘连蛋白5是一种异源三聚体基底膜蛋白,对真皮-表皮连接的结构和功能至关重要。它由三个糖蛋白亚基组成:α3、β3和γ2链,分别由LAMA3、LAMB3和LAMC2基因编码。这些基因中的任何一个发生突变都会导致遗传性交界性大疱性表皮松解症(JEB)。在美国鞍马驹中,患有皮肤起泡病症上皮形成不全的个体中,发现LAMA3基因存在一个跨越外显子24 - 27的6589碱基对缺失。该缺失证实了美国鞍马中的这种常染色体隐性病症确实可归类为JEB,且与人类的赫利茨型JEB相对应。开发了一种诊断测试,在2007年出生的175匹随机选择的美国鞍马驹中,有9匹被发现是该突变的携带者(频率为0.026)。

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