Production and disposal of medium-chain fatty acids in children with medium-chain acyl-CoA dehydrogenase deficiency
| Autor: | D. Halliday, James V. Leonard, G. N. Thompson, A. F. Massoud, Simon Heales, Shamima Rahman |
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| Rok vydání: | 1994 |
| Předmět: |
Male
medicine.medical_specialty Lipolysis Dehydrogenase Fatty Acids Nonesterified Biology Acyl-CoA Dehydrogenase Acyl-CoA Dehydrogenases In vivo Internal medicine Genetics medicine Humans Child Genetics (clinical) chemistry.chemical_classification Fatty Acids Decanoates Infant Acyl CoA dehydrogenase Fasting Metabolism Medium-Chain Acyl-CoA Dehydrogenase Deficiency In vitro Endocrinology Enzyme chemistry biology.protein Female Caprylates Oxidation-Reduction |
| Zdroj: | Journal of Inherited Metabolic Disease. 17:74-80 |
| ISSN: | 1573-2665 0141-8955 |
| DOI: | 10.1007/bf00735398 |
| Popis: | The effect of fasting on plasma concentrations of fatty acids has been determined in four children with medium-chain acyl-CoA dehydrogenase (MCAD) deficiency. In addition, the in vivo rate of octanoate oxidation was measured, using [1-13C]octanoate. In the three older children (1.5-11.2 years), fasting for up to 18 h stimulated lipolysis, as reflected by the increasing concentration of free fatty acids, but with little rise in concentrations of medium-chain fatty acids, octanoate, decanoate and cis-4-decenoate. In an infant (0.5 year), lipolysis was greater and was accompanied by rising concentrations of medium-chain fatty acids. After 13.5 h there was a rapid increase in the concentration of decanoate and cis-4-decenoate. The calculated in vivo rate of octanoate oxidation was substantial in all patients studied (6.4-13.1 mumol/kg per h) despite very low MCAD activity in vitro. It is concluded that under basal conditions the in vivo oxidation rate of medium-chain fatty acids is near normal in the four children studied with MCAD deficiency. |
| Databáze: | OpenAIRE |
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