A new humanized ataxin-3 knock-in mouse model combines the genetic features, pathogenesis of neurons and glia and late disease onset of SCA3/MJD

Neurobiology of Disease

Volumn 73, Issue , 2015, Pages 174-188

  Switonski, Pawel M ^a   Szlachcic, Wojciech J ^a   Krzyzosiak, Wlodzimierz J ^a   Figiel, Maciej ^a  

^a INSTITUTE OF BIOORGANIC CHEMISTRY   (Poland)

Author keywords

Ataxia; Ataxin 3; CAG repeats; Knock in; Knockin; MJD; Mouse; Polyglutamine; SCA3; Serpina3n; Spinocerebellar

Indexed keywords

ATAXIN 3; CALBINDIN 1; RAUWOLFIA ALKALOID; SERPINA3N PROTEIN; UNCLASSIFIED DRUG; ATXN3 PROTEIN, HUMAN; NERVE PROTEIN; NUCLEAR PROTEIN; REPRESSOR PROTEIN;

AGE; ALLELE; ANIMAL CELL; ANIMAL EXPERIMENT; ANIMAL MODEL; ANIMAL TISSUE; ARTICLE; ASTROCYTOSIS; ATAXIN 3 GENE; BIOACCUMULATION; CAG REPEAT; CELL COUNT; CEREBELLUM; CONTROLLED STUDY; DISEASE MARKER; FEMALE; GENE MUTATION; GENE TARGETING; GENETIC ASSOCIATION; GENOMIC INSTABILITY; GLIA; HUMAN; HUMAN CELL; IMMUNOHISTOCHEMISTRY; MALE; MOUSE; NERVE CELL; NERVOUS SYSTEM INFLAMMATION; NEUROPATHOLOGY; NONHUMAN; PHENOTYPE; PONS; PROTEIN EXPRESSION; PROTEIN LOCALIZATION; PURKINJE CELL; ROTAROD TEST; SPINOCEREBELLAR ATAXIA TYPE 3; SPINOCEREBELLAR DEGENERATION; SUBSTANTIA NIGRA; TESTIS; TISSUE SPECIFICITY; WHITE MATTER; ANIMAL; BRAIN; CELL NUCLEUS INCLUSION BODY; COMPLICATION; DISEASE MODEL; ENCEPHALITIS; GENE EXPRESSION REGULATION; GENETICS; MACHADO JOSEPH DISEASE; METABOLISM; PATHOLOGY; SENSATION DISORDERS; TRANSGENIC MOUSE; TRINUCLEOTIDE REPEAT;

ANIMALS; BRAIN; CALBINDIN 1; DISEASE MODELS, ANIMAL; ENCEPHALITIS; FEMALE; GENE EXPRESSION REGULATION; HUMANS; INTRANUCLEAR INCLUSION BODIES; MACHADO-JOSEPH DISEASE; MALE; MICE; MICE, TRANSGENIC; NERVE TISSUE PROTEINS; NEUROGLIA; NEURONS; NUCLEAR PROTEINS; REPRESSOR PROTEINS; SENSATION DISORDERS; TRINUCLEOTIDE REPEATS;

EID: 84908701336     PISSN: 09699961     EISSN: 1095953X     Source Type: Journal    
DOI: 10.1016/j.nbd.2014.09.020     Document Type: Article

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