短指伴新型BMP8A和FGFR1变异:一例报告并文献复习

Luke Hunter, Muhammad Ilyas
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引用次数: 0

摘要

骨形态发生蛋白(BMPs)和成纤维细胞生长因子受体1 (FGFR1)基因在骨骼系统的发育和维持中发挥重要作用。短指畸形是一种遗传性疾病,其特征是手和脚的骨头缩短或缺失。已经确定了几种类型的短指畸形,每种都与不同的基因突变有关。然而,有些病例不适合现有的分类,需要进一步的遗传调查。我们对一名34岁女性患者的左脚第二指中指骨缺失和她13岁的儿子的十个脚趾中指骨缺失或畸形进行了评估。全基因组测序(WGS)分析在BMP8A基因中发现了一个错义变体(c.1073A >;T; p.K358M),在FGFR1基因中发现了一个新的错义变体(c.1787C >;T, p.Ser596Phe)。功能蛋白关联网络分析显示BMP8A和FGFR1与其他短指致病基因有很强的相关性。鉴于这些突变之前没有与任何已知的短指亚型联系起来,它们可能定义了一种独特的遗传状况。研究结果提出了一种新型的短指畸形,命名为AB型短指畸形。
本文章由计算机程序翻译,如有差异,请以英文原文为准。

Brachydactyly with Novel BMP8A and FGFR1 Variants: A Case Report with Review of Literature

Brachydactyly with Novel BMP8A and FGFR1 Variants: A Case Report with Review of Literature

Bone morphogenetic proteins (BMPs) and the fibroblast growth factor receptor 1 (FGFR1) gene play essential roles in the development and maintenance of the skeletal system. Brachydactyly is a genetic condition characterized by shortened or missing bones in the hands and feet. Several types of brachydactyly have been identified, each associated with different genetic mutations. However, some cases do not fit into existing classifications, necessitating further genetic investigation. A 34-year-old female patient with an absent middle phalanx in the second digit of her left foot and her 13-year-old son, who presented with absent or malformed middle and distal phalanx in all ten toes, are evaluated. Whole Genome Sequencing (WGS) analysis identifies a missense variant (c.1073A >T; p.K358M) in the BMP8A gene and a novel missense variant (c.1787C >T, p.Ser596Phe) in the FGFR1 gene. Functional protein association network analysis demonstrates a strong association of BMP8A and FGFR1 with other brachydactyly disease-causing genes. Given that these mutations have not been previously linked to any recognized brachydactyly subtype, they likely define a distinct genetic condition. The findings suggest a novel form of brachydactyly, which naming is proposed as brachydactyly type AB.

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