Title: 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.
Publication Year: 1991
Publication Date: 1991-01-29
Language: en
Type: article
Indexed In: ['crossref', 'pubmed']
Access and Citation
Cited By Count: 3
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