| Literature DB >> 29441481 |
Guoyan Qi1, Peng Liu2, Shanshan Gu2, Hongxia Yang2, Huimin Dong2, Yinping Xue2.
Abstract
Our study is aimed to explore the underlying genetic basis of myasthenia gravis. We collected a Chinese pedigree with myasthenia gravis, and whole-exome sequencing was performed on the two affected siblings and their parents. The candidate pathogenic gene was identified by bioinformatics filtering, which was further verified by Sanger sequencing. The homozygous mutation c.G40A (p.V14M) in interferon gamma receptor 1was identified. Moreover, the mutation was also detected in 3 cases of 44 sporadic myasthenia gravis patients. The p.V14M substitution in interferon gamma receptor 1 may affect the signal peptide function and the translocation on cell membrane, which could disrupt the binding of the ligand of interferon gamma and antibody production, contributing to myasthenia gravis susceptibility. We discovered that a rare variant c.G40A in interferon gamma receptor 1 potentially contributes to the myasthenia gravis pathogenesis. Further functional studies are needed to confirm the effect of the interferon gamma receptor 1 on the myasthenia gravis phenotype.Entities:
Keywords: Autoimmune disease; Interferon gamma receptor 1; Myasthenia gravis; Pathologenesis; Whole-exome sequencing
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Year: 2018 PMID: 29441481 DOI: 10.1007/s10072-018-3275-8
Source DB: PubMed Journal: Neurol Sci ISSN: 1590-1874 Impact factor: 3.307