A transgenic mouse model of spinocerebellar ataxia type 3 resembling late disease onset and gender-specific instability of CAG repeats

Neurobiology of Disease

Volumn 37, Issue 2, 2010, Pages 284-293

  Boy, Jana ^a   Schmidt, Thorsten ^a   Schumann, Ulrike ^a   Grasshoff, Ute ^a   Unser, Samy ^a   Holzmann, Carsten ^b   Schmitt, Ina ^c   Karl, Tim ^d   Laccone, Franco ^e   Wolburg, Hartwig ^f   Ibrahim, Saleh ^b   Riess, Olaf ^a  

^a UNIVERSITY OF TÜBINGEN   (Germany)

^b UNIVERSITY OF ROSTOCK   (Germany)

^c UNIVERSITY OF BONN   (Germany)

^d HANNOVER MEDICAL SCHOOL   (Germany)

^e MEDICAL UNIVERSITY OF VIENNA   (Austria)

^f UNIVERSITY HOSPITAL TÜBINGEN   (Germany)

Author keywords

CAG repeat instability; Intranuclear inclusion bodies; Late onset; Machado Joseph disease; MJD; Polyglutamine; SCA3; Spinocerebellar ataxia type 3; Transgenic mouse model

Indexed keywords

ADENINE; ATAXIN 3; CYTOSINE; GUANINE; HUNTINGTIN;

ANIMAL EXPERIMENT; ANIMAL MODEL; ANIMAL TISSUE; ARTICLE; CONTROLLED STUDY; DISEASE COURSE; DISEASE TRANSMISSION; GENDER; GENETIC STABILITY; HYPERACTIVITY; MACHADO JOSEPH DISEASE; MOTOR COORDINATION; MOUSE; NERVE CELL LESION; NONHUMAN; ONSET AGE; PRIORITY JOURNAL; PROGENY; PROMOTER REGION; PROTEIN AGGREGATION; PROTEIN EXPRESSION; SYMPTOM; TRANSGENIC MOUSE; UBIQUITINATION;

AGE OF ONSET; ANIMALS; BRAIN; CHROMOSOMAL INSTABILITY; DISEASE MODELS, ANIMAL; DISEASE PROGRESSION; GENE EXPRESSION; GENETIC PREDISPOSITION TO DISEASE; INTRANUCLEAR INCLUSION BODIES; MACHADO-JOSEPH DISEASE; MICE; MICE, TRANSGENIC; MOVEMENT DISORDERS; MUTATION; NUCLEAR PROTEINS; RATS; SEX CHARACTERISTICS; TRANSCRIPTION FACTORS; TRINUCLEOTIDE REPEATS;

EID: 72749118732     PISSN: 09699961     EISSN: None     Source Type: Journal    
DOI: 10.1016/j.nbd.2009.08.002     Document Type: Article

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