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伊朗异染性脑白质营养不良患者中芳基硫酸酯酶 A 基因突变谱。

Spectrum of ARSA mutations in Iranian patients with metachromatic leukodystrophy.

作者信息

Fathi Mohadeseh, Khalilian Sheyda, Miryounesi Mohammad, Ghafouri-Fard Soudeh

机构信息

Student Research Committee, School of Medicine, Shahid Beheshti University of Medical Sciences, Tehran, Iran.

Department of Medical Genetics, Shahid Beheshti University of Medical Sciences, Tehran, Iran.

出版信息

Biochem Genet. 2025 Jan 13. doi: 10.1007/s10528-025-11025-2.

Abstract

Metachromatic leukodystrophy (MLD) is an autosomal recessive disorder caused by mutations in the arylsulfatase A (ARSA) gene. Few studies have assessed the spectrum of ARSA mutations among Iranian patients. Here, we report eight Iranian patients with clinical features of MLD. Whole exome sequencing led to identification of the underlying mutation in ARSA gene in these patients. Among identified mutations was the recurrent c.938G > C (p.R313P) mutation in exon 5 of this gene, showing its relatively high frequency among Iranians. The results of this study helps in design of population-specific panels for screening purposes in order to decrease the burden of MLD.

摘要

异染性脑白质营养不良(MLD)是一种常染色体隐性疾病,由芳基硫酸酯酶A(ARSA)基因突变引起。很少有研究评估伊朗患者中ARSA突变的范围。在此,我们报告8例具有MLD临床特征的伊朗患者。全外显子组测序确定了这些患者ARSA基因的潜在突变。在已识别的突变中,该基因第5外显子中反复出现的c.938G>C(p.R313P)突变,表明其在伊朗人群中频率相对较高。本研究结果有助于设计针对特定人群的筛查检测板,以减轻MLD的负担。

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