Tags

Type your tag names separated by a space and hit enter

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

ten Brink HJ
Department of Paediatrics, Free University Hospital, Amsterdam, The Netherlands.
Poll-The BT
No affiliation info available
Saudubray JM
No affiliation info available
Wanders RJ
No affiliation info available
Jakobs C
No affiliation info available

MeSH

Acyl-CoA OxidaseFatty AcidsHumansMicrobodiesOxidation-ReductionOxidoreductasesPhytanic Acid

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 -