Development of Purkinje cell degeneration in a knockin mouse model reveals lysosomal involvement in the pathogenesis of SCA6

Proceedings of the National Academy of Sciences of the United States of America

Volumn 109, Issue 43, 2012, Pages 17693-17698

  Unno, Toshinori ^a   Wakamori, Minoru ^b   Koike, Masato ^c   Uchiyama, Yasuo ^c   Ishikawa, Kinya ^a   Kubota, Hisahiko ^b   Yoshida, Takashi ^b   Sasakawa, Hiroko ^a   Peters, Christoph ^d   Mizusawa, Hidehiro ^a   Watase, Kei ^a  

^a TOKYO MEDICAL AND DENTAL UNIVERSITY   (Japan)

^b TOHOKU UNIVERSITY   (Japan)

^c JUNTENDO UNIVERSITY GRADUATE SCHOOL OF MEDICINE   (Japan)

^d UNIVERSITY OF FREIBURG   (Germany)

Author keywords

Cerebellum; Inherited ataxia; P Q type calcium channel

Indexed keywords

CATHEPSIN B; CYSTEINE PROTEINASE; MEMBRANE PROTEIN; POLYGLUTAMINE; VOLTAGE GATED CALCIUM CHANNEL;

ANIMAL EXPERIMENT; ANIMAL MODEL; ARTICLE; AUTOPHAGY; BIOACCUMULATION; CELL DEGENERATION; CELL MEMBRANE; CEREBELLUM; CONTROLLED STUDY; ENDOPLASMIC RETICULUM; EXPERIMENTAL MODEL; IMMUNOFLUORESCENCE; LYSOSOME; MOUSE; NONHUMAN; PATHOGENESIS; PRIORITY JOURNAL; PROMOTER REGION; PROTEIN DEGRADATION; PROTEIN EXPRESSION; PROTEIN FUNCTION; PROTEIN LOCALIZATION; PURKINJE CELL; QUANTITATIVE ANALYSIS; REVERSE TRANSCRIPTION POLYMERASE CHAIN REACTION; SPINOCEREBELLAR ATAXIA TYPE 6; SPINOCEREBELLAR DEGENERATION;

ANIMALS; AUTOPHAGY; DISEASE MODELS, ANIMAL; LYSOSOMES; MICE; MICE, TRANSGENIC; PURKINJE CELLS; SPINOCEREBELLAR ATAXIAS;

EID: 84867908880     PISSN: 00278424     EISSN: 10916490     Source Type: Journal    
DOI: 10.1073/pnas.1212786109     Document Type: Article

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