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鉴定 CFTR 基因中的超罕见 Alu 插入:遗传检测解读中的陷阱和挑战。

Identification of an ultra-rare Alu insertion in the CFTR gene: Pitfalls and challenges in genetic test interpretation.

机构信息

Department of Molecular Medicine and Medical Biotechnologies, University of Naples Federico II, Naples, Italy; CEINGE- Advanced Biotechnologies Franco Salvatore, Naples, Italy.

CEINGE- Advanced Biotechnologies Franco Salvatore, Naples, Italy; Department of Translational Medical Science, University of Campania Luigi Vanvitelli, Naples, Italy.

出版信息

Clin Chim Acta. 2024 May 15;558:118317. doi: 10.1016/j.cca.2024.118317. Epub 2024 Apr 4.

Abstract

Cystic fibrosis (CF) is a life-limiting genetic disorder characterized by defective chloride ion transport due to mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene. Early detection through newborn screening programs significantly improves outcomes for individuals with CF by enabling timely intervention. Here, we report the identification of an Alu element insertion within the exon 15 of CFTR gene, initially overlooked in standard next-generation sequencing analyses. However, using traditional molecular techniques, based on polymerase chain reaction and Sanger sequencing, allowed the identification of the Alu element and the reporting of a correct diagnosis. Our analysis, based on bioinformatics tools and molecular techniques, revealed that the Alu element insertion severely affects the gene expression, splicing patterns, and structure of CFTR protein. In conclusion, this study emphasizes the importance of how the integration of human expertise and modern technologies represents a pivotal step forward in genomic medicine, ensuring the delivery of precision healthcare to individuals affected by genetic diseases.

摘要

囊性纤维化 (CF) 是一种具有生命限制的遗传性疾病,其特征是由于囊性纤维化跨膜电导调节因子 (CFTR) 基因突变导致氯离子转运缺陷。通过新生儿筛查计划早期发现,通过及时干预,显著改善了 CF 患者的预后。在这里,我们报告了在 CFTR 基因的外显子 15 内发现一个 Alu 元件插入,该插入最初在标准下一代测序分析中被忽略。然而,使用基于聚合酶链反应和 Sanger 测序的传统分子技术,使我们能够鉴定 Alu 元件并报告正确的诊断。我们的分析基于生物信息学工具和分子技术,揭示了 Alu 元件插入严重影响 CFTR 基因的表达、剪接模式和结构。总之,这项研究强调了人类专业知识与现代技术相结合的重要性,这是基因组医学向前迈出的关键一步,确保为受遗传疾病影响的个体提供精准医疗。

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