Generation of induced pluripotent stem cell line (CSUXHi002-A) from a patient with spinocerebellar ataxia type 1

Lang He; Huifang Zhao; Shuai Li; Xiaobo Han; Zhao Chen; Chunrong Wang; Chao Tian; Feng Tang; Rongqi Huang; Zuoxian Lin; Zhiyuan Li; Beisha Tang; Hong Jiang

Stem Cell Research (May 2020)

Generation of induced pluripotent stem cell line (CSUXHi002-A) from a patient with spinocerebellar ataxia type 1

Lang He,
Huifang Zhao,
Shuai Li,
Xiaobo Han,
Zhao Chen,
Chunrong Wang,
Chao Tian,
Feng Tang,
Rongqi Huang,
Zuoxian Lin,
Zhiyuan Li,
Beisha Tang,
Hong Jiang

Affiliations

Lang He: Department of Neurology, Xiangya Hospital, Central South University, Changsha, Hunan, China
Huifang Zhao: School of Life Sciences, University of Science and Technology of China, Hefei, China
Shuai Li: University of Chinese Academy of Sciences, Beijing, China
Xiaobo Han: University of Chinese Academy of Sciences, Beijing, China
Zhao Chen: Department of Neurology, Xiangya Hospital, Central South University, Changsha, Hunan, China
Chunrong Wang: Department of Neurology, Xiangya Hospital, Central South University, Changsha, Hunan, China
Chao Tian: School of Life Sciences, University of Science and Technology of China, Hefei, China
Feng Tang: University of Chinese Academy of Sciences, Beijing, China
Rongqi Huang: CAS Key Laboratory of Regenerative Biology, Guangdong Provincial Key Laboratory of Stem Cell. and Regenerative Medicine, Guangzhou Institutes of Biomedicine and Health, Chinese Academy of Sciences, Guangzhou, China
Zuoxian Lin: CAS Key Laboratory of Regenerative Biology, Guangdong Provincial Key Laboratory of Stem Cell. and Regenerative Medicine, Guangzhou Institutes of Biomedicine and Health, Chinese Academy of Sciences, Guangzhou, China
Zhiyuan Li: School of Life Sciences, University of Science and Technology of China, Hefei, China; University of Chinese Academy of Sciences, Beijing, China; CAS Key Laboratory of Regenerative Biology, Guangdong Provincial Key Laboratory of Stem Cell. and Regenerative Medicine, Guangzhou Institutes of Biomedicine and Health, Chinese Academy of Sciences, Guangzhou, China; GZMU-GIBH Joint School of Life Sciences, Guangzhou Medical University, Guangzhou, China; Department of Anatomy and Neurobiology, Xiangya School of Medicine, Central South. University, Changsha, Hunan, China
Beisha Tang: Department of Neurology, Xiangya Hospital, Central South University, Changsha, Hunan, China; National Clinical Research Center for Geriatric Diseases, Central South University, Changsha, Hunan, China; Key Laboratory of Hunan Province in Neurodegenerative Disorders, Central South University, Changsha, Hunan, China; Laboratory of Medical Genetics, Central South University, Changsha, Hunan, China
Hong Jiang: Department of Neurology, Xiangya Hospital, Central South University, Changsha, Hunan, China; National Clinical Research Center for Geriatric Diseases, Central South University, Changsha, Hunan, China; Key Laboratory of Hunan Province in Neurodegenerative Disorders, Central South University, Changsha, Hunan, China; Laboratory of Medical Genetics, Central South University, Changsha, Hunan, China; Xinjiang Medical University, Urumchi, Xinjiang, China; Corresponding author.

Journal volume & issue: Vol. 45

Abstract

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Spinocerebellar ataxia type 1 (SCA1) is a hereditary neurodegenerative disease caused by CAG repeated expansion in ATXN1 gene. We generated induced pluripotent stem cells (iPSCs) from the urine exfoliated epithelial cells of SCA1 patient by using the integration-free methods. The patient derived iPSCs retained the mutation (the 65 CAG expansion tracts in ATXN1 gene), displayed normal karyotypes, expressed pluripotency markers and had the potential to differentiate towards three germ layers in vivo. This type of stem cell model will be valuable for elucidating the pathological mechanism and screening potential drugs of SCA1.

Published in Stem Cell Research

ISSN: 1873-5061 (Print); 1876-7753 (Online)
Publisher: Elsevier
Country of publisher: Netherlands
LCC subjects: Science: Biology (General)
Website: https://www.journals.elsevier.com/stem-cell-research

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