Lethal mitochondrial cardiomyopathy in a hypomorphic Med30 mouse mutant is ameliorated by ketogenic diet

Proceedings of the National Academy of Sciences of the United States of America

Volumn 108, Issue 49, 2011, Pages 19678-19682

  Krebs, Philippe ^a,f   Fan, Weiwei ^b   Chen, Yen Hui ^c   Tobita, Kimimasa ^d   Downes, Michael R ^b   Wood, Malcolm R ^a   Sun, Lei ^a   Li, Xiaohong ^a   Xia, Yu ^a   Ding, Ning ^b   Spaeth, Jason M ^e   Moresco, Eva Marie Y ^a   Boyer, Thomas G ^e   Lo, Cecilia Wen Ya ^d   Yen, Jeffrey ^c   Evans, Ronald M ^b   Beutler, Bruce ^a,g  

^a SCRIPPS RESEARCH INSTITUTE   (United States)

^b HOWARD HUGHES MEDICAL INSTITUTE   (United States)

^c INSTITUTE OF BIOMEDICAL SCIENCES   (Taiwan)

^d CHILDREN S HOSPITAL OF PITTSBURGH   (United States)

^e UNIVERSITY OF TEXAS HEALTH SCIENCE CENTER AT SAN ANTONIO   (United States)

^f UNIVERSITY OF BERN   (Switzerland)

^g UNIVERSITY OF TEXAS AT DALLAS   (United States)

Author keywords

Heart; Metabolism; Peroxisome proliferator activated receptor coactivator 1

Indexed keywords

ANIMAL EXPERIMENT; ANIMAL TISSUE; ARTICLE; CARDIOMYOPATHY; FATTY ACID OXIDATION; GENE; GENE EXPRESSION; GENE FUNCTION; GENETIC TRANSCRIPTION; HEART FAILURE; HOMOZYGOSITY; KETOGENIC DIET; LACTATION; MED30 GENE; MISSENSE MUTATION; MITOCHONDRION; MOUSE; MUTATIONAL ANALYSIS; NONHUMAN; OXIDATIVE PHOSPHORYLATION; PHENOTYPE; PLEIOTROPY; PRIORITY JOURNAL; WEANING;

AMINO ACID SEQUENCE; ANIMALS; BASE SEQUENCE; CARDIOMYOPATHIES; ELECTROPHORESIS, POLYACRYLAMIDE GEL; FEMALE; GENE EXPRESSION; GENES, LETHAL; KAPLAN-MEIER ESTIMATE; KETOGENIC DIET; MALE; MEDIATOR COMPLEX; MICE; MICE, INBRED C3H; MICE, INBRED C57BL; MICROSCOPY, ELECTRON; MITOCHONDRIA, HEART; MITOCHONDRIAL MYOPATHIES; MUTATION, MISSENSE; MYOCARDIUM; PROTEIN SUBUNITS; REVERSE TRANSCRIPTASE POLYMERASE CHAIN REACTION; SEQUENCE HOMOLOGY, AMINO ACID; SEQUENCE HOMOLOGY, NUCLEIC ACID; WEANING;

EID: 83755195189     PISSN: 00278424     EISSN: 10916490     Source Type: Journal    
DOI: 10.1073/pnas.1117835108     Document Type: Article

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