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一例具有单纯型大疱性表皮松解症的杂交牛犊中 KRT5 的从头突变。

A de novo mutation in KRT5 in a crossbred calf with epidermolysis bullosa simplex.

机构信息

Department of Veterinary Medical Sciences, University of Bologna, Bologna, Italy.

Institute of Genetics, Vetsuisse Faculty, University of Bern, Bern, Switzerland.

出版信息

J Vet Intern Med. 2020 Nov;34(6):2800-2807. doi: 10.1111/jvim.15943. Epub 2020 Nov 2.

Abstract

A 6-day-old Belgian Blue-Holstein calf was referred because of a syndrome resembling epidermolysis bullosa simplex (EBS). The clinical phenotype included irregular and differently sized erosions and ulcerations spread over the body, in particular on the limbs and over bone prominences, as well as in the nasal planum and oral mucosa. Blisters were easily induced by rubbing the skin. The skin lesions displayed a clear dermal-epidermal separation at the level of the basal cell layer. Post mortem examination revealed erosions in the pharynx, proximal esophagus, and rumen. Whole-genome sequencing revealed a heterozygous disruptive in-frame deletion variant in KRT5 (c.534_536delCAA). Genotyping of both parents confirmed the variant as de novo mutation. Clinicopathological and genetic findings were consistent with the diagnosis of KRT5-related EBS providing the second example of a spontaneous mutation causing epidermolysis bullosa in cattle.

摘要

一只 6 日龄的比利时蓝白-荷斯坦牛犊因类似于单纯型大疱性表皮松解症(EBS)的综合征而被转介。临床表型包括散布在全身的不规则和大小不一的糜烂和溃疡,特别是在四肢和骨突上,以及在鼻平面和口腔黏膜上。摩擦皮肤很容易引起水疱。皮肤病变在基底层水平显示出明显的表皮-真皮分离。剖检显示咽、食管近端和瘤胃有糜烂。全基因组测序显示 KRT5 中存在杂合性破坏性框内缺失变异(c.534_536delCAA)。对父母双方的基因分型证实该变异为新生突变。临床病理和遗传发现与 KRT5 相关 EBS 的诊断一致,为牛中自发性突变引起大疱性表皮松解症的第二个例子提供了依据。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/cdf1/7694802/c229fcc3637e/JVIM-34-2800-g001.jpg

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