Pristanic acid does not accumulate in peroxisomal acyl-CoA oxidase deficiency: evidence for a distinct peroxisomal pristanyl-CoA oxidase.

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.

• 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)

Chemical References

• Fatty Acids
• Phytanic Acid
• pristanic acid
• Oxidoreductases
• pristanoyl-CoA oxidase
• Acyl-CoA Oxidase

Topics

• Acyl-CoA Oxidase
• Fatty Acids (metabolism)
• Humans
• Microbodies (enzymology)
• Oxidation-Reduction
• Oxidoreductases (deficiency, metabolism)
• Phytanic Acid (blood)