Clinical features, neurogenetics and neuropathology of the polyglutamine spinocerebellar ataxias type 1, 2, 3, 6 and 7

Progress in Neurobiology

Volumn 104, Issue , 2013, Pages 38-66

  Rüb, Udo ^a   Schöls, Ludger ^b   Paulson, Henry ^c   Auburger, Georg ^a   Kermer, Pawel ^d   Jen, Joanna C ^e   Seidel, Kay ^a   Korf, Horst Werner ^a   Deller, Thomas ^a  

^a GOETHE UNIVERSITY   (Germany)

^b UNIVERSITY OF TÜBINGEN   (Germany)

^c UNIVERSITY OF MICHIGAN   (United States)

^d UNIVERSITY OF GÖTTINGEN   (Germany)

^e UNIVERSITY OF CALIFORNIA   (United States)

Author keywords

ADCA; Ataxia; Neurodegeneration; Neurogenetics; Neuropathology; Polyglutamine diseases

Indexed keywords

ATAXIN 1; ATAXIN 2; ATAXIN 3; ATAXIN 6; ATAXIN 7; NEUROTROPHIC FACTOR; POLYGLUTAMINE; UNCLASSIFIED DRUG;

ASTROCYTE; BRAIN ELECTROPHYSIOLOGY; BRAIN INJURY; BRAIN REGION; CAG REPEAT; CLINICAL FEATURE; DEMYELINATION; DISEASE SEVERITY; ELECTROOCULOGRAPHY; EVOKED BRAIN STEM AUDITORY RESPONSE; EVOKED MUSCLE RESPONSE; EVOKED SOMATOSENSORY RESPONSE; GAIN OF FUNCTION MUTATION; GENETIC ANALYSIS; GENETIC SCREENING; GENOTYPE; GLIOSIS; GLOMERULONEPHRITIS; HUMAN; HYPERTROPHIC CARDIOMYOPATHY; KIDNEY TUBULE ACIDOSIS; MACROPHAGE; MICROGLIA; MOLECULAR INTERACTION; NERVE DEGENERATION; NEUROPATHOLOGY; NEUROPROTECTION; NONHUMAN; NUCLEAR MAGNETIC RESONANCE IMAGING; OLIGODENDROGLIA; OXIDATIVE STRESS; PATHOGENESIS; PATHOPHYSIOLOGY; PRIORITY JOURNAL; REVIEW; SPINOCEREBELLAR DEGENERATION;

ANIMALS; BRAIN; HUMANS; NERVE TISSUE PROTEINS; NEUROGLIA; PEPTIDES; SPINOCEREBELLAR ATAXIAS;

EID: 84876783597     PISSN: 03010082     EISSN: 18735118     Source Type: Journal    
DOI: 10.1016/j.pneurobio.2013.01.001     Document Type: Review

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