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Pristanic acid does not accumulate in peroxisomal acyl-CoA oxidase deficiency: evidence for a distinct peroxisomal pristanyl-CoA oxidase.
J Inherit Metab Dis. 1991; 14(5):681-4.JI

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+Show Affiliations

Department of Paediatrics, Free University Hospital, Amsterdam, The Netherlands.No affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Journal Article

Language

eng

PubMed ID

1779614

Citation

ten Brink, H J., et al. "Pristanic Acid Does Not Accumulate in Peroxisomal acyl-CoA Oxidase Deficiency: Evidence for a Distinct Peroxisomal pristanyl-CoA Oxidase." Journal of Inherited Metabolic Disease, vol. 14, no. 5, 1991, pp. 681-4.
ten Brink HJ, Poll-The BT, Saudubray JM, et al. Pristanic acid does not accumulate in peroxisomal acyl-CoA oxidase deficiency: evidence for a distinct peroxisomal pristanyl-CoA oxidase. J Inherit Metab Dis. 1991;14(5):681-4.
ten Brink, H. J., Poll-The, B. T., Saudubray, J. M., Wanders, R. J., & Jakobs, C. (1991). Pristanic acid does not accumulate in peroxisomal acyl-CoA oxidase deficiency: evidence for a distinct peroxisomal pristanyl-CoA oxidase. Journal of Inherited Metabolic Disease, 14(5), 681-4.
ten Brink HJ, et al. Pristanic Acid Does Not Accumulate in Peroxisomal acyl-CoA Oxidase Deficiency: Evidence for a Distinct Peroxisomal pristanyl-CoA Oxidase. J Inherit Metab Dis. 1991;14(5):681-4. PubMed PMID: 1779614.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Pristanic acid does not accumulate in peroxisomal acyl-CoA oxidase deficiency: evidence for a distinct peroxisomal pristanyl-CoA oxidase. AU - ten Brink,H J, AU - Poll-The,B T, AU - Saudubray,J M, AU - Wanders,R J, AU - Jakobs,C, PY - 1991/1/1/pubmed PY - 1991/1/1/medline PY - 1991/1/1/entrez SP - 681 EP - 4 JF - Journal of inherited metabolic disease JO - J Inherit Metab Dis VL - 14 IS - 5 N2 - 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. SN - 0141-8955 UR - https://www.unboundmedicine.com/medline/citation/1779614/Pristanic_acid_does_not_accumulate_in_peroxisomal_acyl_CoA_oxidase_deficiency:_evidence_for_a_distinct_peroxisomal_pristanyl_CoA_oxidase_ DB - PRIME DP - Unbound Medicine ER -