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164 名无关联波兰患者的杜氏/贝克肌营养不良症中的小突变。

Small mutations in Duchenne/Becker muscular dystrophy in 164 unrelated Polish patients.

机构信息

Department of Genetics, Institute of Psychiatry and Neurology, 02-957, Sobieskiego 9, Warsaw, Poland.

, Warsaw, Poland.

出版信息

J Appl Genet. 2021 May;62(2):289-295. doi: 10.1007/s13353-020-00605-0. Epub 2021 Jan 9.

Abstract

In the 164 patients with Duchenne/Becker muscular dystrophy, we found 142 different small mutations including 51 novel mutations not listed in the LOVD, the UMD-DMD, the ClinVar, and the HGMD databases. Among all mutations, nonsense mutations occurred in 45.7%, frameshift mutations in 32.9%, and splicing mutations in 19.5%. Small mutations were distributed throughout the whole dystrophin gene. Splicing mutations were twice more common in BMD patients than in DMD patients. Eighty-two percent of mothers of the males affected with DMD/BMD were found to be carriers of small mutations.

摘要

在 164 名杜氏/贝克型肌营养不良症患者中,我们发现了 142 种不同的小突变,包括 51 种新的突变,这些突变未在 LOVD、UMD-DMD、ClinVar 和 HGMD 数据库中列出。在所有突变中,无义突变占 45.7%,移码突变占 32.9%,剪接突变占 19.5%。小突变分布在整个 dystrophin 基因中。剪接突变在 BMD 患者中比在 DMD 患者中更为常见,是后者的两倍。82%的 DMD/BMD 男性患者的母亲被发现是小突变的携带者。

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