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

@article{Ishida2016VulnerabilityOP,
  title={Vulnerability of Purkinje Cells Generated from Spinocerebellar Ataxia Type 6 Patient-Derived iPSCs.},
  author={Yoshihito Ishida and Hideshi Kawakami and Hiroyuki Kitajima and Ayaka Nishiyama and Yoshiki Sasai and Haruhisa Inoue and Keiko Muguruma},
  journal={Cell reports},
  year={2016},
  volume={17 6},
  pages={1482-1490}
}
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 mechanism and effective therapeutic treatments are still unknown. Here, we have succeeded in generating differentiated Purkinje cells that carry patient genes by combining disease-specific iPSCs and… CONTINUE READING
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