Normalization of the Tricaprin Test for ω-Oxidation in Refsum's Disease upon Lowering of Serum Phytanic Acid
- 1 January 1967
- journal article
- research article
- Published by Taylor & Francis in Scandinavian Journal of Clinical and Laboratory Investigation
- Vol. 20 (4) , 294-296
- https://doi.org/10.3109/00365516709076958
Abstract
The ability of patients with Refsum's disease to ω-oxidize fatty acids has been followed by the tricaprin test during the course of lowering serum phytanic acid by dietary treatment. At high levels of phytanic acid accumulation, the test reveals an interference with the ω-oxidation system. Lowering of the phytanic acid normalizes the test, indicating that the primary cause of phytanic acid accumulation is not to be found in the ω-oxidizing system.Keywords
This publication has 10 references indexed in Scilit:
- α-Oxidation as an alternative pathway for the degradation of branched-chain fatty acids in man, and its failure in patients with refsum's diseaseBiochimica et Biophysica Acta (BBA) - Lipids and Lipid Metabolism, 1967
- Studies on the Metabolic Error in Refsum's Disease*Journal of Clinical Investigation, 1967
- Alpha-decarboxylation, an important pathway for degradation of phytanic acid in animalsBiochemical and Biophysical Research Communications, 1966
- The existence of an alternative pathway for the degradation of branch-chained fatty acids, and its failure in heredopathia atactica polyneuriti-formis (Refsum's disease)Biochimica et Biophysica Acta (BBA) - Lipids and Lipid Metabolism, 1966
- DIETARY EFFECTS ON SERUM-PHYTANIC-ACID LEVELS AND ON CLINICAL MANIFESTATIONS IN HEREDOPATHIA ATACTICA POLYNEURITIFORMISThe Lancet, 1966
- The Ability of Patients with Heredopathia Atactica Poly-Neukitiformis to ω-Oxidize and Degrade Several Isoprenoid Branch-Chained Fatty StructuresScandinavian Journal of Clinical and Laboratory Investigation, 1966
- Alpha-Oxidation of Branched Chain Fatty Acids in Man and its Failure in Patients with Refsum's Disease Showing Phytanic Acid AccumulationScandinavian Journal of Clinical and Laboratory Investigation, 1966
- Conversion of U-C14-phytol to phytanic acid and its oxidation in heredopathia atactica polyneuritiformisBiochemical and Biophysical Research Communications, 1965
- Über das Vorkommen der 3.7.11.15-Tetramethyl-hexadecansäure (Phytansäure) in den Cholesterinestern und anderen Lipoidfraktionen der Organe bei einem Krankheitsfall unbekannter Genese (Verdacht auf Heredopathia atactica polyneuritiformis [Refsum-Syndrom])Hoppe-Seyler´s Zeitschrift Für Physiologische Chemie, 1963
- Researches on fat metabolism: XII. The Influence of carbohydrate on diaciduriaBiochimica et Biophysica Acta, 1948