Impaired peroxisomal fatty acid oxidation in human skin fibroblasts with a mitochondrial acylcarnitine/carnitine translocase deficiency

Journal of Inherited Metabolic Disease

Volumn 19, Issue 2, 1996, Pages 185-187

  Jakobs, B S ^a   Wanders, R J A ^a  

^a ACADEMIC MEDICAL CENTER   (Netherlands)

Author keywords

[No Author keywords available]

Indexed keywords

ACYLCARNITINE CARNITINE TRANSLOCASE; CARRIER PROTEIN; UNCLASSIFIED DRUG;

CONFERENCE PAPER; CONTROLLED STUDY; CULTURE MEDIUM; ENZYME DEFICIENCY; FATTY ACID OXIDATION; HUMAN; HUMAN CELL; MITOCHONDRION; PATHOGENESIS; PEROXISOME; SKIN FIBROBLAST;

CARNITINE ACYLTRANSFERASES; FATTY ACIDS; FIBROBLASTS; HUMANS; MICROBODIES; MITOCHONDRIA; OXIDATION-REDUCTION; PALMITIC ACID; PALMITIC ACIDS; SKIN;

EID: 0029880033     PISSN: 01418955     EISSN: None     Source Type: Journal    
DOI: 10.1007/BF01799425     Document Type: Conference Paper

References (4)

1. Jakobs BS, Wanders RJA (1995) Fatty acid oxidation in peroxisomes and mitochondria: the first, unequivocal evidence for the involvement of carnitine in shuttling propionyl-CoA from peroxisomes to mitochondria. Biochem Biophys Res Commun 213: 1035-1041.
2. Pande SV, Brivet M, Slama A, Demaugre F, Aufrant C, Saudubray JM (1993) Carnitine/acylcarnitine translocase deficiency with severe hypoglycemia and auriculo-ventricular block. J Clin Invest 91: 1247-1252.
3. Pollitt RJ ( 1995) Disorders of mitochondrial long-chain fatty acids oxidation. J Inher Metab Dis 18: 473-490.
4. Reddy JK, Mannaerts GP (1994) Peroxisomal lipid metabolism. Annu Rev Nutr 14: 343-370.