Vulnerability of Purkinje Cells Generated from Spinocerebellar Ataxia Type 6 Patient-Derived iPSCs
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- 作者：Yoshihito Ishida1 ; 2 ; 3 ; Hideshi Kawakami4 ; Hiroyuki Kitajima1 ; Ayaka Nishiyama2 ; Yoshiki Sasai1 ; 6 ; Haruhisa Inoue5 ; Keiko Muguruma1 ; 2 ; 7 ; muguruma@cdb.riken.jp" class="auth_mail" title="E-mail the corresponding author
- 关键词：spinocerebellar ataxia type 6 ; SCA6 ; Purkinje cell ; induced pluripotent stem cells ; iPSCs ; disease modeling
- 刊名：Cell Reports
- 出版年：2016
- 出版时间：1 November 2016
- 年：2016
- 卷：17
- 期：6
- 页码：1482-1490
- 全文大小：4822 K
文摘
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Purkinje cells are generated from SCA6 patient-derived iPSCs
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Patient cells show increased full-length Cav2.1, but decreased C-terminal fragment
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T3 depletion causes decrease in cell number and dendritic arbors in patient cells
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T3 depletion-induced degeneration was suppressed by treatment with TRH or Riluzole