Regulation of mitochondrial fatty acid β-oxidation in human: What can we learn from inborn fatty acid β-oxidation deficiencies?

Biochimie

Volumn 96, Issue 1, 2014, Pages 113-120

  Bastin, Jean ^a  

^a INSERM   (France)

Author keywords

Fibrates; Inborn enzyme defects; Mitochondrial fatty acid oxidation; Newborn screening; Pharmacological therapy; PPAR

Indexed keywords

FATTY ACID; LONG CHAIN FATTY ACID; MEDIUM CHAIN ACYL COENZYME A DEHYDROGENASE; PEROXISOME PROLIFERATOR ACTIVATED RECEPTOR ALPHA; PEROXISOME PROLIFERATOR ACTIVATED RECEPTOR GAMMA COACTIVATOR 1ALPHA;

CHEMICAL ANALYSIS; DRUG TARGETING; ENZYME DEFECT; ESSENTIAL FATTY ACID DEFICIENCY; FATALITY; FATTY ACID OXIDATION; GENE MUTATION; GENETIC HETEROGENEITY; HUMAN; LOSS OF FUNCTION MUTATION; MITOCHONDRION; MULTIPLE ORGAN FAILURE; PHENOTYPE; RARE DISEASE; REGULATORY MECHANISM; REVIEW;

FIBRATES; INBORN ENZYME DEFECTS; MITOCHONDRIAL FATTY ACID Β-OXIDATION; NEWBORN SCREENING; PHARMACOLOGICAL THERAPY; PPAR;

ACYL-COA DEHYDROGENASE; ANIMALS; FATTY ACIDS; HUMANS; LIPID METABOLISM, INBORN ERRORS; MITOCHONDRIA; MOLECULAR TARGETED THERAPY; OXIDATION-REDUCTION;

EID: 84890472757     PISSN: 03009084     EISSN: 61831638     Source Type: Journal    
DOI: 10.1016/j.biochi.2013.05.012     Document Type: Review

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