Evidence of mitochondrial dysfunction in fragile X-associated tremor/ataxia syndrome

Biochemical Journal

Volumn 429, Issue 3, 2010, Pages 545-552

  Ross Inta, Catherine ^a   Omanska Klusek, Alicja ^a   Wong, Sarah ^a   Barrow, Cedrick ^a   Garcia Arocena, Dolores ^b   Iwahashi, Christine ^b   Berry Kravis, Elizabeth ^c   Hagerman, Randi J ^b,d   Hagerman, Paul J ^a,d   Giulivi, Cecilia ^a  

^a UNIVERSITY OF CALIFORNIA   (United States)

^b UNIVERSITY OF CALIFORNIA   (United States)

^c RUSH UNIVERSITY MEDICAL CENTER   (United States)

^d UNIVERSITY OF CALIFORNIA   (United States)

Author keywords

Fragile X associated tremor ataxia syndrome (FXTAS); Mitochondrion; Neurodegeneration; Oxidative stress; Trinucleotide repeat expansion; Unfolded protein response

Indexed keywords

ADDITIONAL TREATMENT; BRAIN SAMPLE; ELECTRON TRANSPORT; ENVIRONMENTAL STRESSORS; FRAGILE X-ASSOCIATED TREMOR/ATAXIA SYNDROME (FXTAS); MENTAL RETARDATION; MITOCHONDRIAL DYSFUNCTION; MITOCHONDRIAL PROTEIN; NEURODEGENERATION; NEURODEGENERATIVE DISORDERS; OXYGEN UPTAKE RATES; PARKINSON'S DISEASE; PATHOLOGICAL PROCESS; TRINUCLEOTIDE REPEAT EXPANSION; UNFOLDED PROTEIN RESPONSE; UNTRANSLATED REGIONS;

CELL CULTURE; EXPANSION; FIBROBLASTS; HANDICAPPED PERSONS; MITOCHONDRIA; OXYGEN;

PROTEINS;

ADENOSINE TRIPHOSPHATE; FLAVINE ADENINE NUCLEOTIDE; MITOCHONDRIAL PROTEIN; NICOTINAMIDE ADENINE DINUCLEOTIDE; FMR1 PROTEIN, HUMAN; FRAGILE X MENTAL RETARDATION PROTEIN;

ARTICLE; CELL DEGENERATION; ELECTRON TRANSPORT; ENZYME ACTIVITY; ENZYME SYNTHESIS; FRIEDREICH ATAXIA; GENE MUTATION; HUMAN; HUMAN CELL; HUMAN TISSUE; MALE; MITOCHONDRION; NERVE DEGENERATION; OXIDATIVE STRESS; OXYGEN CONSUMPTION; PRIORITY JOURNAL; PROTEIN EXPRESSION; TRANSLATION REGULATION; ADULT; AGED; ATAXIA; BIOSYNTHESIS; FRAGILE X SYNDROME; GENETICS; MIDDLE AGED; MUTATION; PATHOPHYSIOLOGY; PHYSIOLOGY; PROTEIN SYNTHESIS; TREMOR;

ADENOSINE TRIPHOSPHATE; ADULT; AGED; AGED, 80 AND OVER; ATAXIA; ELECTRON TRANSPORT; FRAGILE X MENTAL RETARDATION PROTEIN; FRAGILE X SYNDROME; HUMANS; MIDDLE AGED; MITOCHONDRIA; MUTATION; PROTEIN BIOSYNTHESIS; TREMOR;

EID: 77955071201     PISSN: 02646021     EISSN: 14708728     Source Type: Journal    
DOI: 10.1042/BJ20091960     Document Type: Article

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