14q23.2 微缺失或 AKAP5 单倍缺失可能是导致智力障碍的潜在原因吗?

IF 1.5 4区 医学 Q4 GENETICS & HEREDITY Psychiatric Genetics Pub Date : 2024-06-01 Epub Date: 2024-04-20 DOI:10.1097/YPG.0000000000000368
Fayize Maden Bedel, Özgür Balasar, Ayşe Şimşek, Hüseyin Tokgöz, Hüseyin Çaksen
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引用次数: 0

摘要

智障的特征是在以下领域中至少有两个领域存在障碍:社交技能、沟通技能、自理 任务和学习技能。这些障碍的评估是根据个人的年龄和文化水平,对照预期标准进行的。此外,智力障碍通常是指智力功能的可测量水平,以智商核心值 70 或以下为代表。自闭症谱系障碍是一种因大脑差异导致的发育障碍,通常表现为社交沟通和互动方面的问题,以及行为或兴趣的局限性或重复性。遗传性球形红细胞增多症是一种以贫血、黄疸和脾脏肿大为特征的疾病,由于细胞膜蛋白的遗传性损伤,红细胞从具有中心苍白的双凹圆盘形细胞形态转变为缺乏中心苍白的球形细胞,从而导致溶血倾向增加。一名 11 岁的女性患者因发育迟缓和遗传性球形红细胞症的诊断而被转诊至儿科遗传学分科。由于她还具有畸形的面部特征,如额部隆起、前额宽而突出、管状鼻结构和朱砂薄,因此进行了基因检测。染色体微阵列分析显示,14q23.2q23.3 区域存在 2.5 Mb 的缺失。在她的父亲身上也发现了相同区域的缺失,他也有相同的表型特征,包括遗传性球形红细胞增多症和学习困难。我们认为,位于该区域的 PLEKHG3 和 AKAP5 基因可能会导致智力障碍的发生。
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Could the 14q23.2 microdeletion or AKAP5 haploinsufficiency be a potential cause of intellectual disability?

Intellectual disability is characterized by impairment in at least two of the following areas: social skills, communication skills, self-care tasks, and academic skills. These impairments are evaluated in relation to the expected standards based on the individual's age and cultural levels. Additionally, intellectual disability is typically defined by a measurable level of intellectual functioning, represented by an intelligence quotients core of 70 or below. Autism spectrum disorder is a developmental disability resulting from differences in the brain, often characterized by problems in social communication and interaction, and limited or repetitive behaviors or interests. Hereditary spherocytosis is a disease characterized by anemia, jaundice, and splenomegaly as a result of increased tendency to hemolysis with morphological transformation of erythrocytes from biconcave disc-shaped cells with central pallor to spherocytes lacking central pallor due to hereditary injury of cellular membrane proteins. An 11-year-old female patient was referred to Pediatric Genetics Subdivision due to the presence of growth retardation and a diagnosis of hereditary spherocytosis. Since she also had dysmorphic facial features, such as frontal bossing, broad and prominent forehead, tubular nasal structure, and thin vermillion, genetic tests were performed. Chromosomal microarray analysis revealed a 2.5 Mb deletion in the 14q23.2q23.3 region. Deletion was also identified in the same region in her father, who had the same phenotypic characteristics, including hereditary spherocytosis and learning difficulties. We propose that the PLEKHG3 and AKAP5 genes, which are located in this region, may contribute to the development of intellectual disability.

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来源期刊
Psychiatric Genetics
Psychiatric Genetics 医学-神经科学
CiteScore
2.30
自引率
0.00%
发文量
39
审稿时长
3 months
期刊介绍: ​​​​​​The journal aims to publish papers which bring together clinical observations, psychological and behavioural abnormalities and genetic data. All papers are fully refereed. Psychiatric Genetics is also a forum for reporting new approaches to genetic research in psychiatry and neurology utilizing novel techniques or methodologies. Psychiatric Genetics publishes original Research Reports dealing with inherited factors involved in psychiatric and neurological disorders. This encompasses gene localization and chromosome markers, changes in neuronal gene expression related to psychiatric disease, linkage genetics analyses, family, twin and adoption studies, and genetically based animal models of neuropsychiatric disease. The journal covers areas such as molecular neurobiology and molecular genetics relevant to mental illness. Reviews of the literature and Commentaries in areas of current interest will be considered for publication. Reviews and Commentaries in areas outside psychiatric genetics, but of interest and importance to Psychiatric Genetics, will also be considered. Psychiatric Genetics also publishes Book Reviews, Brief Reports and Conference Reports.
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