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FYCO1 基因中的一个新突变导致先天性白内障:一个中国家庭的病例研究。

A Novel Mutation in the FYCO1 Gene Causing Congenital Cataract: Case Study of a Chinese Family.

机构信息

Department of Ophthalmology, Xiang'an Hospital of Xiamen University, Eye Institute & Affiliated Xiamen Eye Center, School of Medicine, Xiamen University, Xiamen 361102, China.

Fujian Provincial Key Laboratory of Ophthalmology and Visual Science, School of Medicine, Xiamen University, Xiamen 361102, China.

出版信息

Dis Markers. 2022 Aug 26;2022:5838104. doi: 10.1155/2022/5838104. eCollection 2022.

Abstract

Congenital cataract is the most important global cause of visual impairment in children. Autosomal dominant and autosomal recessive inheritance account for the majority of the hereditary nonsyndromic congenital cataract. The function of FYCO1 gene is to guide the transport of the microtubule-directed vesicles. Mutations in the FYCO1 gene may cause cataracts. We reported a novel nonsense mutation in FYCO1 (c.1411C > T, P. R471 ∗), which could cause nonsyndrome autosomal recessive congenital cataract. We underwent an ophthalmology examination of all participants and collected blood samples from all participants and extracted genomic DNAs. By whole exome sequencing, we found that this family carried an unreported mutation in the FYCO1 gene: c.1411C > T, P. R471 ∗. Sanger sequencing was performed to verify the mutation. We used ITASSER and PYMOL to predict and compare the structure and function of the mutated proteins. Using SIFT software and referring to the relevant guidelines of ACMG, the mutation was determined to be pathogenic. The models suggested that the nonsense mutation p.R471∗ resulted in a profound disruption of the FYCO1 protein structure. This report expands the locus information of the FYCO1 mutations.

摘要

先天性白内障是全球儿童视力损害的最重要原因。常染色体显性遗传和常染色体隐性遗传占遗传性非综合征性先天性白内障的大多数。FYCO1 基因的功能是指导微管定向囊泡的运输。FYCO1 基因的突变可能导致白内障。我们报道了 FYCO1 中的一个新的无义突变(c.1411C > T,P. R471∗),它可能导致常染色体隐性非综合征性先天性白内障。我们对所有参与者进行了眼科检查,并采集了所有参与者的血液样本并提取了基因组 DNA。通过全外显子组测序,我们发现该家系携带 FYCO1 基因中一个未报道的突变:c.1411C > T,P. R471∗。进行 Sanger 测序以验证突变。我们使用 ITASSER 和 PYMOL 来预测和比较突变蛋白的结构和功能。使用 SIFT 软件并参考 ACMG 的相关指南,确定该突变是致病性的。模型表明,无义突变 p.R471∗导致 FYCO1 蛋白结构的严重破坏。本报告扩展了 FYCO1 突变的基因座信息。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/85c1/9439885/f97f27b7f4de/DM2022-5838104.001.jpg

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