Proteomics reveals that redox regulation is disrupted in patients with ethylmalonic encephalopathy

Journal of Proteome Research

Volumn 10, Issue 5, 2011, Pages 2389-2396

  Palmfeldt, Johan ^a   Vang, Søren ^a   Stenbroen, Vibeke ^a   Pavlou, Evangelos ^b   Baycheva, Mila ^c   Buchal, Gebhard ^d   Monavari, Ardeshir Ahmad ^e   Augoustides Savvopoulou, Persephone ^b   Mandel, Hanna ^f   Gregersen, Niels ^a  

^a AARHUS UNIVERSITY HOSPITAL   (Denmark)

^b ARISTOTLE UNIVERSITY OF THESSALONIKI   (Greece)

^c University Pediatric Hospital   (Bulgaria)

^d Hospital of Kirchen   (Germany)

^e CHILDREN S UNIVERSITY HOSPITAL   (Ireland)

^f TECHNION ISRAEL INSTITUTE OF TECHNOLOGY   (Israel)

Author keywords

ethylmalonic encephalopathy; H2S; HSCO; oxidative stress; quantitative proteomics

Indexed keywords

ALDEHYDE DEHYDROGENASE; ALDEHYDE DEHYDROGENASE X; APOPTOSIS INDUCING FACTOR; CHLORIDE INTRACELLULAR CHANNEL 4; DIMETHYLARGININE DIMETHYLAMINOHYDROLASE 1; GENE PRODUCT; HYDROLASE; LACTATE DEHYDROGENASE; MANGANESE SUPEROXIDE DISMUTASE; NITRIC OXIDE; OXIDOREDUCTASE; PROTEIN AIFM1; PROTEIN ETHE1; SULFIDE; SULFIDE:QUINONE OXIDOREDUCTASE; UNCLASSIFIED DRUG;

ARTICLE; CONTROLLED STUDY; DETOXIFICATION; DISEASE SEVERITY; ENERGY METABOLISM; ENZYME ACTIVITY; ETHYLMALONIC ENCEPHALOPATHY; FIBROBLAST; HUMAN; HUMAN CELL; ION TRANSPORT; METABOLIC DISORDER; MITOCHONDRION; NUCLEOTIDE SEQUENCE; OXIDATION REDUCTION REACTION; PRIORITY JOURNAL; PROTEOMICS; REDOX STRESS; SYMPTOM;

ALDEHYDE DEHYDROGENASE; AMIDOHYDROLASES; APOPTOSIS INDUCING FACTOR; BRAIN DISEASES, METABOLIC, INBORN; CELLS, CULTURED; CHLORIDE CHANNELS; CHROMATOGRAPHY, LIQUID; FIBROBLASTS; GENE EXPRESSION REGULATION; HUMANS; L-LACTATE DEHYDROGENASE; MITOCHONDRIA; OXIDATION-REDUCTION; PROTEOMICS; PURPURA; SKIN; SULFIDES; SUPEROXIDE DISMUTASE; TANDEM MASS SPECTROMETRY;

EID: 79955831977     PISSN: 15353893     EISSN: 15353907     Source Type: Journal    
DOI: 10.1021/pr101218d     Document Type: Article

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