| Literature DB >> 31325086 |
Zuobin Zhu1, Xiaoxiao Han2, Ying Li3, Conghui Han4, Mengqiong Deng5, Yuhao Zhang6, Qing Shen5, Yijuan Cao5,7, Zhenbei Li5,7, Xitao Wang4, Juan Gu5,7, Xiaoyan Liu5,7, Yaru Yang5,7, Qiang Zhang8, Fangfang Hu9,10.
Abstract
Kallmann syndrome (KS) is a congenital hypogonadotropic hypogonadism that coincides with anosmia or hyposmia. Although this rare genetic disease has a very low incidence, it harbors a complicated genetic heterogeneity, which indicates X-linked, autosomal, and oligogenic inheritance of puberty, sexuality, reproductivity, and olfactory defects. There has been limited elucidation of molecular etiologies completed to date. Here, a chromosome reciprocal translocation (46, XX, t (3; 13) (p13; q22)) was identified in a 27-year-old Chinese female diagnosed with KS. Genome sequencing found an intronic breakpoint of SCEL in chromosome 13 and an intergenic breakpoint between ROBO1 and ROBO2 in chromosome 3. This translocation resulted in the reduced expression levels of these genes. An array-CGH test captured no abnormal genomic copy numbers of clinical significance. The basic features of all known KS-related genes were also reviewed and analyzed for their roles in KS onset with bioinformatic methods. Signal pathway and gene enrichment analysis of KS-related genes suggested that these genes have integrated functions in neuronal migration and differentiation. An interesting chromosome locational pattern of KS-related genes was also discovered. This study provided constructive clues for further investigations into the molecular etiology of KS.Entities:
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Year: 2019 PMID: 31325086 DOI: 10.1007/s12020-019-02010-y
Source DB: PubMed Journal: Endocrine ISSN: 1355-008X Impact factor: 3.633