Wojciechowska Katarzyna, Zie Whitley, Pietrzyk Aleksandra, Lejman Monika
Independent Laboratory of Genetic Diagnostics, Medical University, Lublin, Poland.
Student Scientific Society, Independent Laboratory of Genetic Diagnostics, Medical University Lublin, Poland.
Ann Agric Environ Med. 2024 Jun 27;31(2):306-310. doi: 10.26444/aaem/171758. Epub 2023 Sep 22.
The NAA10 gene encodes N-alpha-acetyltransferase 10 which plays an important role in cell growth, differentiation, DNA damage, metastasis, apoptosis, stress response and autophagy. Defects in the NAA10 gene correlate with the diagnosis of NAA10-related syndrome (Ogden syndrome). The most common symptoms of NAA10-related syndrome are: global developmental delay, non-verbal or limited speech, autism spectrum disorder, feeding difficulties, motor delay, muscle tone disturbances, and long QT syndrome. To-date, there are about 100 patients who have been reported with this condition. The case report presents the clinical study of a girl aged 4 years and 3 months diagnosed with Ogden syndrome. She had many characteristic features of the disorder, as well as precocious puberty. This girl represents the case of a patient with p.Arg83Cys mutation in NAA10 gene as well as precocious puberty.
NAA10基因编码N-α-乙酰转移酶10,该酶在细胞生长、分化、DNA损伤、转移、凋亡、应激反应和自噬中发挥重要作用。NAA10基因缺陷与NAA10相关综合征(奥格登综合征)的诊断相关。NAA10相关综合征最常见的症状是:全面发育迟缓、非语言或语言能力有限、自闭症谱系障碍、喂养困难、运动发育迟缓、肌张力障碍和长QT综合征。迄今为止,已有约100例这种疾病的患者被报道。该病例报告展示了一名4岁3个月大被诊断为奥格登综合征女孩的临床研究。她具有该疾病的许多特征性表现,以及性早熟。这名女孩代表了一名NAA10基因存在p.Arg83Cys突变且伴有性早熟的患者病例。