Contribution of odd-chain fatty acid oxidation to propionate production in disorders of propionate metabolism
| Autor: | J M Saudubray, A Mariotti, G N Thompson, F Poggi, D Sbaï, C Narcy, J L Bresson |
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| Přispěvatelé: | Laboratoire de Biogéochimie Isotopique, Université Pierre et Marie Curie - Paris 6 (UPMC) |
| Jazyk: | angličtina |
| Rok vydání: | 1994 |
| Předmět: |
medicine.medical_specialty
Adolescent Phenylalanine Methylmalonic acid Medicine (miscellaneous) Models Biological 03 medical and health sciences chemistry.chemical_compound 0302 clinical medicine Lipid oxidation Internal medicine medicine Humans Child Beta oxidation ComputingMilieux_MISCELLANEOUS 030304 developmental biology chemistry.chemical_classification 0303 health sciences Nutrition and Dietetics Catabolism Chemistry Fatty Acids Calorimetry Indirect Metabolism Fasting Endocrinology Child Preschool Propionate Odd-chain fatty acid Propionates Oxidation-Reduction [SDV.AEN]Life Sciences [q-bio]/Food and Nutrition 030217 neurology & neurosurgery Metabolism Inborn Errors |
| Zdroj: | American Journal of Clinical Nutrition American Journal of Clinical Nutrition, American Society for Nutrition, 1994, 59, pp.1332-1337 The American Journal of Clinical Nutrition The American Journal of Clinical Nutrition, 1994, 59, pp.1332-1337 |
| ISSN: | 0002-9165 1938-3207 |
| Popis: | Whole-body propionate and protein kinetics and energy substrate metabolism were studied in five metronidazole-treated patients with propionic or methylmalonic acidemias by the use of a primed, 4-h constant infusion of [1-13C]propionate and L-[O-2H5]phenylalanine combined with indirect calorimetry. Measurements were performed during fasting and carbohydrate feeding, successively, to assess the contribution of odd-chain fatty acid oxidation to total propionate production. Fat oxidation decreased from 490 +/- 179 to 57 +/- 49 mumol.kg-1.h-1 (P < 0.05) as a result of feeding. Propionate appearance rate was 38.6 +/- 8 mumol.kg-1.h-1 during fasting and decreased to 22.6 +/- 5 mumol.kg-1.h-1 (P < 0.05) on the carbohydrate diet. Precursor amino acid catabolism did not change significantly (22 +/- 5 vs 21.2 +/- 5 mumol.kg-1.h-1), suggesting that the 41% reduction in propionate production observed in response to feeding was related to the suppression of fatty acid oxidation. Therefore, significant therapeutic gains may be expected from the use of diets aimed at reducing lipid oxidation. |
| Databáze: | OpenAIRE |
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