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3q13.33 - 3q21.2微缺失:神经发育障碍的罕见病因

Microdeletion 3q13.33-3q21.2: A Rare Cause of Neurodevelopmental Disorder.

作者信息

Huang Yi Juan, Jia Rong Pu, Chen Yuan Qiu, Zhou Liang Ji, Gou Chen Yu, Fan Mei Qiong, Li Si, Chen Maofa, Lin Hua Ming, Gao Yu

机构信息

Department of Obstetrics and Gynecology, Sixth Affiliated Hospital of Sun Yat-Sen University, Guangzhou, China.

Biomedical Innovation Center, The Sixth Affiliated Hospital, Sun Yat-Sen University, Guangzhou, China.

出版信息

J Pediatr Genet. 2024 Jul 4;13(4):283-290. doi: 10.1055/s-0044-1788031. eCollection 2024 Dec.

Abstract

Chromosomal sub-microscopic imbalances, such as microdeletions and microduplications, are associated with multiple genetic disorders. Here, we illustrate microdeletion 3q13.33q21.2 might be responsible for neurodevelopmental disorder in two patients. There are two patients with neurodevelopmental disorder in a family of seven. We used chromosomal microarray analysis to identify the microdeletion 3q13.33q21.2. Next-generation sequencing was utilized to exclude the presence of allelic mutations within the microdeletion region 3q13.33q21.2, which may have a potential role in the development of disease in patients affected with secondary genetic alterations. Patient 4 was diagnosed with dilated left third ventricle, neurodevelopmental disorder, and mild abnormalities in electroencephalogram through a series of clinical examinations. Patient 6 was diagnosed with attention deficit hyperactivity disorder, short stature, intellectual disability, and concurrent epilepsy. By investigating the 3q13.33q21.2 band of the University of California, Santa Cruz database, we screened out the genes related to developmental delay and intellectual disability, including ADCY5 SEMA5B andKPNA1, which were highly suspected to be related to intelligence. This region also involves CASR, a gene that has been reported to be associated with epilepsy. The ADCY5 and SEMA5B genes may be key genes to cause neurodevelopmental disorder. Abnormal expression of the CASR gene may lead to the occurrence of epilepsy.

摘要

染色体亚显微失衡,如微缺失和微重复,与多种遗传疾病相关。在此,我们阐明微缺失3q13.33q21.2可能是两名患者神经发育障碍的病因。在一个七口之家有两名患有神经发育障碍的患者。我们使用染色体微阵列分析来鉴定微缺失3q13.33q21.2。利用下一代测序来排除微缺失区域3q13.33q21.2内等位基因突变的存在,这些突变可能在受继发性基因改变影响的患者疾病发展中起潜在作用。通过一系列临床检查,患者4被诊断为左侧第三脑室扩张、神经发育障碍和脑电图轻度异常。患者6被诊断为注意力缺陷多动障碍、身材矮小、智力残疾和并发癫痫。通过研究加利福尼亚大学圣克鲁兹分校数据库的3q13.33q21.2带,我们筛选出了与发育迟缓及智力残疾相关的基因,包括ADCY5、SEMA5B和KPNA1,这些基因被高度怀疑与智力有关。该区域还涉及CASR,一个据报道与癫痫相关的基因。ADCY5和SEMA5B基因可能是导致神经发育障碍的关键基因。CASR基因的异常表达可能导致癫痫的发生。

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