Formation of 2,3-pristenic acid and 3-hydroxypristanic acid from pristanic acid in human liver

Journal of Inherited Metabolic Disease

Volumn 20, Issue 3, 1997, Pages 441-443

  Verhoeven, N M ^a   Schor, D S M ^a   Jansen, G A ^b   Kok, R M ^a   Ten Brink, H J ^a   Wanders, R J A ^b   Jakobs, C ^a  

^a VU UNIVERSITY MEDICAL CENTER   (Netherlands)

^b UNIVERSITY OF AMSTERDAM   (Netherlands)

Author keywords

[No Author keywords available]

Indexed keywords

2,3 PRISTENIC ACID; 3 HYDROXYPRISTANIC ACID; COENZYME A; FATTY ACID; PRISTANIC ACID; UNCLASSIFIED DRUG;

CONFERENCE PAPER; CONTROLLED STUDY; FATTY ACID OXIDATION; FATTY ACID SYNTHESIS; GAS CHROMATOGRAPHY; HUMAN; HUMAN TISSUE; HYDROLYSIS; IONIZATION; LIVER; MASS FRAGMENTOGRAPHY; PEROXISOME; ZELLWEGER SYNDROME;

ADENOSINE TRIPHOSPHATE; COENZYME A; FATTY ACIDS; FATTY ACIDS, UNSATURATED; HUMANS; LIVER; MAGNESIUM; OXIDATION-REDUCTION; ZELLWEGER SYNDROME;

EID: 0030791246     PISSN: 01418955     EISSN: None     Source Type: Journal    
DOI: 10.1023/A:1005375222568     Document Type: Conference Paper

References (5)

1. Rasmussen JT, Börchers T, Knutsen J (1990) Comparison of the binding affinities of acyl-CoA-binding protein and fatty acid binding protein for long-chain acyl-CoA esters. Biochem J 265: 849-855.
2. Ten Brink HJ, Stellaard F, van den Heuvel CMM, et al (1992) Pristanic acid and phytanic acid in plasma from patients with peroxisomal disorders: stable isotope dilution analysis with electron capture negative ion mass fragmentography. J Lipid Res 33: 41-47.
3. Wanders RJA, Ten Brink HJ, van Roermund CWT, Schutgens RBH, Tager JM, Jakobs C (1990) Identification of pristanoyl-CoA oxidase activity in human liver and its deficiency in the Zellweger syndrome. Biochem Biophys Res Commun 172: 490-495.
4. Wanders RJA, Van Roermund CWT, Schor DSM, ten Brink HJ, Jakobs C (1994) 2-Hydroxyphytanic acid oxidase activity in rat and human liver and its deficiency in the Zellweger syndrome. Biochim Biophys Acta 1227: 177-182.
5. Wanders RJA, Denis S, Ruiter JPN, Schutgens RBH, Van Roermund CWT, Jakobs BS (1995) Measurement of peroxisomal fatty acid β-oxidation in cultured human skin fibroblasts. J Inher Metab Dis 18 (supplement 1): 113-124.