Abstract |
The concentration of pristanic acid was measured in plasma from a patient with an isolated peroxisomal very long chain fatty acid (VLCFA) acyl-CoA oxidase deficiency, a defect in peroxisomal beta-oxidation resulting in accumulation of VLCFA in plasma and tissues. Although peroxisomes are believed to be involved in pristanic acid beta-oxidation, the pristanic acid level in the patient's plasma was within the control range. This finding provides evidence for the existence of a pristanyl-CoA oxidase distinct from the specific trihydroxycholestanoyl- CoA and VLCFA acyl-CoA oxidases.
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Authors | H J ten Brink, B T Poll-The, J M Saudubray, R J Wanders, C Jakobs |
Journal | Journal of inherited metabolic disease
(J Inherit Metab Dis)
Vol. 14
Issue 5
Pg. 681-4
( 1991)
ISSN: 0141-8955 [Print] United States |
PMID | 1779614
(Publication Type: Journal Article)
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Chemical References |
- Fatty Acids
- Phytanic Acid
- pristanic acid
- Oxidoreductases
- pristanoyl-CoA oxidase
- Acyl-CoA Oxidase
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Topics |
- Acyl-CoA Oxidase
- Fatty Acids
(metabolism)
- Humans
- Microbodies
(enzymology)
- Oxidation-Reduction
- Oxidoreductases
(deficiency, metabolism)
- Phytanic Acid
(blood)
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