DRPLA transgenic mouse substrains carrying single copy of full-length mutant human DRPLA gene with variable sizes of expanded CAG repeats exhibit CAG repeat length- and age-dependent changes in behavioral abnormalities and gene expression profiles

DRPLA transgenic mouse substrains carrying single copy of full-length mutant human DRPLA gene with variable sizes of expanded CAG repeats exhibit CAG repeat length- and age-dependent changes in behavioral abnormalities and gene expression profiles

Dentatorubral-pallidoluysian atrophy (DRPLA) is an autosomal dominant progressive neurodegenerative disorder with intellectual deterioration and various motor deficits including ataxia, choreoathetosis, and myoclonus, caused by an abnormal expansion of CAG repeats in the DRPLA gene. Longer expanded...

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Bibliographic Details
Main Authors: Kazushi Suzuki, Jiayi Zhou, Toshiya Sato, Keizo Takao, Tsuyoshi Miyagawa, Mutsuo Oyake, Mitunori Yamada, Hitoshi Takahashi, Yuji Takahashi, Jun Goto, Shoji Tsuji
Format: Article
Language:English
Published: Elsevier 2012-05-01
Series:Neurobiology of Disease
Subjects:
Dentatorubral-pallidoluysian atrophy
DRPLA transgenic mice
Neurodegeneration
Polyglutamine
Expression profiling
Microarray
Online Access:http://www.sciencedirect.com/science/article/pii/S0969996112000381

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