Genomics and Molecular Medicine Division, CSIR - Institute of Genomics and Integrative Biology, New Delhi 110007, India; Academy of Scientific and Innovative Research (AcSIR), Ghaziabad 201002, India.
Genomics and Molecular Medicine Division, CSIR - Institute of Genomics and Integrative Biology, New Delhi 110007, India.
Stem Cell Res. 2024 Apr;76:103319. doi: 10.1016/j.scr.2024.103319. Epub 2024 Jan 26.
The PPP2R2B gene, expressed highly in the brain, harbours trinucleotide CAG repeats in the 5'UTR region, in the range of 7-42 repeats. Individuals carrying CAG repeats greater than 43 have been associated to manifest a neurodegenerative disease condition termed as Spinocerebellar Ataxia type 12 (SCA12). An iPSC line from an adult male diagnosed with SCA12 presenting symptoms of gait (Gait Dominance) was generated. It showed pluripotency and trilineage markers without any chromosomal abnormality. This line can be utilized as an essential resource in enhancing our understanding of the molecular pathogenic mechanisms underlying SCA12 by facilitating generation of various neuronal cell types.
PPP2R2B 基因在大脑中高度表达,其 5'UTR 区域存在三核苷酸 CAG 重复序列,重复次数在 7-42 次之间。携带 CAG 重复次数大于 43 的个体与表现出一种称为脊髓小脑共济失调 12 型(SCA12)的神经退行性疾病状况相关。从一名患有 SCA12 并表现出步态(步态主导)症状的成年男性中生成了一个 iPSC 系。它表现出多能性和三系标志物,没有任何染色体异常。该系可通过促进各种神经元细胞类型的生成,作为增强我们对 SCA12 分子发病机制理解的重要资源。