Washington University School of Medicine, St. Louis, MO 63110, USA.
Department of Pathology and Immunology, Washington University School of Medicine, St. Louis, MO 63110, USA.
Int J Mol Sci. 2022 Apr 12;23(8):4246. doi: 10.3390/ijms23084246.
Hypospadias is a common form of congenital atypical sex development that is often associated with other congenital comorbidities. Many genes have been associated with the condition, most commonly single sequence variations. Further investigations of recurrent and overlapping copy number variations (CNVs) have resulted in the identification of genes and chromosome regions associated with various conditions, including differences of sex development (DSD). In this retrospective study, we investigated the DECIPHER database, as well as an internal institutional database, to identify small recurrent CNVs among individuals with isolated and syndromic hypospadias. We further investigated these overlapping recurrent CNVs to identify 75 smallest regions of overlap (SROs) on 18 chromosomes. Some of the genes within these SROs may be considered potential candidate genes for the etiology of hypospadias and, occasionally, additional comorbid phenotypes. This study also investigates for the first time additional common phenotypes among individuals with hypospadias and overlapping CNVs. This study provides data that may aid genetic counseling and management of individuals with hypospadias, as well as improve understanding of its underlying genetic etiology and human genital development overall.
尿道下裂是一种常见的先天性非典型性性别发育畸形,常伴有其他先天性合并症。许多基因与该疾病有关,最常见的是单一序列变异。对反复出现和重叠的拷贝数变异(CNVs)的进一步研究导致了与各种疾病相关的基因和染色体区域的鉴定,包括性别发育差异(DSD)。在这项回顾性研究中,我们调查了 DECIPHER 数据库以及内部机构数据库,以确定孤立性和综合征性尿道下裂患者中的小重复 CNVs。我们进一步研究了这些重叠的重复 CNVs,以确定 18 条染色体上的 75 个最小重叠区域(SRO)。这些 SRO 中的一些基因可能被认为是尿道下裂病因的潜在候选基因,偶尔还会出现其他合并表型。这项研究还首次调查了尿道下裂和重叠 CNVs 患者的其他常见表型。这项研究提供的数据可能有助于尿道下裂患者的遗传咨询和管理,并提高对其潜在遗传病因和人类生殖器发育的整体理解。