He Xiang, Xie Feng, Ren Zhao-rui
Shanghai Institute of Medical Genetics, Children's Hospital of Shanghai, Shanghai Jiao Tong University School of Medicine, Shanghai, People's Republic of China.
Genet Test Mol Biomarkers. 2012 Apr;16(4):297-301. doi: 10.1089/gtmb.2011.0113. Epub 2012 Feb 17.
Achondroplasia (ACH) is a genetic disorder with autosomal dominant inheritance and is the cause of one of the most common forms of short limb dwarfism in humans. Mutations of special sites in the fibroblast growth factor receptor-3 gene (FGFR3) are reported as a cause of ACH, and almost 98% of cases are caused by mutations in nucleotide 1138 (Gly380Arg), with 97% involving a c.1138G>A mutation and 1% involving a c.1138G>C mutation. Therefore, the development of a simple, reliable, and rapid approach for molecular detection of nucleotide 1138 mutations is of great significance for prevention and early diagnosis of ACH. High-resolution melting (HRM) is a new, rapid, and inexpensive molecular detection method that has been generally applied to mutation scanning. In this study, 12 cases of ACH, including 10 sporadic cases and 2 cases in a pedigree, were detected simultaneously using HRM analysis and restriction fragment length polymorphism-polymerase chain reaction (RFLP-PCR). Eleven cases were identified as carrying the c.1138 G>A heterozygous mutation, and one case was identified as carrying the c.1138 G>C heterozygous mutation. Compared with RFLP-PCR, HRM analysis provided a more rapid, simpler, and less expensive approach for detecting the most common FGFR3 mutations carried by patients with ACH.
软骨发育不全(ACH)是一种常染色体显性遗传的基因紊乱疾病,是人类最常见的短肢侏儒症的病因之一。据报道,成纤维细胞生长因子受体-3基因(FGFR3)特殊位点的突变是ACH的病因,几乎98%的病例是由核苷酸1138(Gly380Arg)的突变引起的,其中97%涉及c.1138G>A突变,1%涉及c.1138G>C突变。因此,开发一种简单、可靠、快速的分子检测方法来检测核苷酸1138突变,对于ACH的预防和早期诊断具有重要意义。高分辨率熔解曲线分析(HRM)是一种新的、快速且廉价的分子检测方法,已普遍应用于突变扫描。在本研究中,使用HRM分析和限制性片段长度多态性-聚合酶链反应(RFLP-PCR)同时检测了12例ACH病例,包括10例散发病例和1个家系中的2例。11例被鉴定为携带c.1138 G>A杂合突变,1例被鉴定为携带c.1138 G>C杂合突变。与RFLP-PCR相比,HRM分析为检测ACH患者携带的最常见FGFR3突变提供了一种更快速、更简单且成本更低的方法。