Carnitine supplementation induces long-chain acylcarnitine production - Studies in the VLCAD-deficient mouse

Journal of Inherited Metabolic Disease

Volumn 29, Issue 2-3, 2006, Pages 343-344

  Liebig, M ^a   Gyenes, M ^a   Brauers, G ^a   Ruiter, J P N ^b   Wendel, U ^a   Mayatepek, E ^a   Strauss, A W ^c   Wanders, R J A ^b   Spiekerkoetter, U ^a  

^a UNIVERSITY HOSPITAL DÜSSELDORF   (Germany)

^b ACADEMIC MEDICAL CENTER   (Netherlands)

^c VANDERBILT UNIVERSITY   (United States)

Author keywords

[No Author keywords available]

Indexed keywords

ACYLCARNITINE; CARNITINE; LONG CHAIN ACYL COENZYME A DEHYDROGENASE;

ANIMAL TISSUE; ARTICLE; BLOOD; COMPARATIVE STUDY; CONTROLLED STUDY; DIET SUPPLEMENTATION; DRUG TISSUE LEVEL; ENZYME DEFICIENCY; EXERCISE; LIPID ANALYSIS; LIPID BLOOD LEVEL; LIPOGENESIS; LIVER; MOUSE; MYOPATHY; NONHUMAN; SKELETAL MUSCLE; TANDEM MASS SPECTROMETRY; TREATMENT OUTCOME; VERY LONG CHAIN ACYL COENZYME A DEHYDROGENASE DEFICIENCY; WILD TYPE;

ACYL-COA DEHYDROGENASE, LONG-CHAIN; ANIMALS; CARNITINE; DIETARY SUPPLEMENTS; DISEASE MODELS, ANIMAL; HOMEOSTASIS; LIPID METABOLISM, INBORN ERRORS; LIVER; MICE; MICE, KNOCKOUT; MUSCLE, SKELETAL; MYOCARDIUM; PHYSICAL CONDITIONING, ANIMAL;

EID: 33745067235     PISSN: 01418955     EISSN: 15732665     Source Type: Journal    
DOI: 10.1007/s10545-006-0249-4     Document Type: Article

References (2)

1. Andresen BS, Olpin S, Poorthuis BJHM, et al (1999) Clear correlation of genotype with disease phenotype in very long-chain acyl-CoA dehydrogenase deficiency. Am J Hum Genet 64: 479-494.
2. Spiekerkoetter U, Tokunaga C, Wendel U, et al (2005) Tissue carnitine homeostasis in very-long-chain acyl-CoA dehydrogenase-deficient mice. Pediatr Res 57: 760-764.