Vulnerability of Purkinje Cells Generated from Spinocerebellar Ataxia Type 6 Patient-Derived iPSCs

Vulnerability of Purkinje Cells Generated from Spinocerebellar Ataxia Type 6 Patient-Derived iPSCs

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Spinocerebellar ataxia type 6 (SCA6) is a dominantly inherited neurodegenerative disease characterized by loss of Purkinje cells in the cerebellum. SCA6 is caused by CAG trinucleotide repeat expansion in CACNA1A, which encodes Cav2.1, α1A subunit of P/Q-type calcium channel. However, the pathogenic...

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Bibliographic Details
Main Authors: Yoshihito Ishida, Hideshi Kawakami, Hiroyuki Kitajima, Ayaka Nishiyama, Yoshiki Sasai, Haruhisa Inoue, Keiko Muguruma
Format: Article
Language:English
Published: Elsevier 2016-11-01
Series:Cell Reports
Subjects:
spinocerebellar ataxia type 6
SCA6
Purkinje cell
induced pluripotent stem cells
iPSCs
disease modeling
Online Access:http://www.sciencedirect.com/science/article/pii/S2211124716314292

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