Human trifunctional protein deficiency: A new disorder of mitochondrial fatty acid β-oridation

Biochemical and Biophysical Research Communications

Volumn 188, Issue 3, 1992, Pages 1139-1145

  Wanders, R J A ^a   Ijlst, L ^a   Poggi, F ^b   Bonnefont, J P ^b   Munnich, A ^b   Brivet, M ^b   Rabier, D ^b   Saudubray, J M ^b  

^a ACADEMIC MEDICAL CENTER   (Netherlands)

^b Section of Radiology   (France)

Author keywords

[No Author keywords available]

Indexed keywords

3 HYDROXYACYL COENZYME A DEHYDROGENASE; ACETYL COENZYME A ACYLTRANSFERASE; DICARBOXYLIC ACID DERIVATIVE; ENOYL COENZYME A HYDRATASE; PALMITIC ACID;

ARTICLE; CASE REPORT; CONTROLLED STUDY; ENZYME ACTIVITY; ENZYME DEFICIENCY; ENZYME MECHANISM; FATTY ACID OXIDATION; GEL FILTRATION; GEL PERMEATION CHROMATOGRAPHY; HUMAN; HUMAN CELL; MALE; METABOLIC DISORDER; MITOCHONDRION; NEWBORN; PRIORITY JOURNAL; SKIN FIBROBLAST; URINE LEVEL;

3-HYDROXYACYL COA DEHYDROGENASES; ACETYL-COA C-ACYLTRANSFERASE; CASE REPORT; CHILD, PRESCHOOL; ENOYL-COA HYDRATASE; FATTY ACIDS; FIBROBLASTS; HUMAN; LIPID METABOLISM, INBORN ERRORS; MITOCHONDRIA; MOLECULAR WEIGHT; MULTIENZYME COMPLEXES; OXIDATION-REDUCTION;

UCHIDA;

EID: 0026458561     PISSN: 0006291X     EISSN: 10902104     Source Type: Journal    
DOI: 10.1016/0006-291X(92)91350-Y     Document Type: Article

References (19)

1. Biochemistry of Peroxisomes
2. Progressive neuropathy and recurrent myoglobinuria in a child with long-chain 3-hydroxyacyl-coenzyme A dehydrogenase deficiency