诱导多能干细胞系 RCPCMi008-A 的建立，该细胞系源自一位脊髓小脑共济失调 17 型患者。
Generation of induced pluripotent stem cell line RCPCMi008-A derived from patient with spinocerebellar ataxia 17.
机构信息
Federal State Budgetary Institution Federal Research and Clinical Center of Physical-Chemical Medicine of Federal Medical Biological Agency, Malaya Pirogovskaya, 1a, 119435 Moscow, Russia.
Federal State Budgetary Institution Federal Research and Clinical Center of Physical-Chemical Medicine of Federal Medical Biological Agency, Malaya Pirogovskaya, 1a, 119435 Moscow, Russia; Lomonosov Moscow State University, GSP-1, Leninskie Gory, 119991 Moscow, Russia.
出版信息
Stem Cell Res. 2021 Jul;54:102431. doi: 10.1016/j.scr.2021.102431. Epub 2021 Jun 16.
IPSC line RCPCMi004-8 was generated from skin fibroblasts collected from a male patient with spinocerebellar ataxia 17. The patient has expanded trinucleotide CAG repeats in the TBP (TATA-binding protein) gene on chromosome 6q27. The reprogramming of fibroblasts was performed with Sendai viruses containing Oct-4, Sox-2, Klf-4, and c-Myc. Pluripotency was confirmed by immunofluorescence, RT-PCR, and the formation of embryoid bodies. The RCPCMi008-A cell line carries the same trinucleotide CAG repeats in the TBP gene. The RCPCMi008-A cell line can be used to model Spinocerebellar ataxia in vitro.
RCPCMi004-8 细胞系源自一位男性脊髓小脑共济失调 17 型患者的皮肤成纤维细胞。该患者在 6q27 染色体上的 TBP（TATA 结合蛋白）基因中存在三核苷酸 CAG 重复扩展。成纤维细胞的重编程是通过含有 Oct-4、Sox-2、Klf-4 和 c-Myc 的仙台病毒进行的。通过免疫荧光、RT-PCR 和类胚体的形成来确认多能性。RCPCMi008-A 细胞系在 TBP 基因中携带相同的三核苷酸 CAG 重复。RCPCMi008-A 细胞系可用于体外模拟脊髓小脑共济失调。
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