Decrease of palmitoyl-CoA elongation in platelets and leukocytes in the patient of hereditary methemoglobinemia associated with mental retardation.
Biochem Biophys Res Commun
; 148(1): 384-91, 1987 Oct 14.
Article
em En
| MEDLINE
| ID: mdl-3675585
ABSTRACT
Effect of the deficiency of NADH-cytochrome b5 reductase on fatty acid elongation was studied in the platelets and leukocytes taken from a patient of hereditary methemoglobinemia associated with mental retardation. The activity of fatty acid elongation was determined by measuring the incorporation of [2-14C]malonyl-CoA into palmitoyl-CoA. The de novo biosynthesis of fatty acids was blocked by the addition of phosphotransacetylase, and the elongation system could be assayed in the homogenates separated from de novo biosynthesis. As compared to normal subjects approximately 40% decrease of fatty acid elongation was observed both in the platelets and leukocytes from the patient.
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Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Palmitoil Coenzima A
/
Plaquetas
/
Acil Coenzima A
/
Leucócitos
/
Deficiência Intelectual
/
Metemoglobinemia
Tipo de estudo:
Etiology_studies
/
Risk_factors_studies
Limite:
Humans
Idioma:
En
Revista:
Biochem Biophys Res Commun
Ano de publicação:
1987
Tipo de documento:
Article