Identification of the newly discovered 58 kDa peroxisomal thiolase SCPx as the main thiolase involved in both pristanic acid and trihydroxycholestanoic acid oxidation: Implications for peroxisomal β-oxidation disorders

Journal of Inherited Metabolic Disease

Volumn 21, Issue 3, 1998, Pages 302-305

  Wanders, R J A ^a,b   Denis, S ^a   Van Berkel, E ^a   Wouters, F ^c   Wirtz, K W A ^c   Seedorf, U ^d  

^a UNIVERSITY OF AMSTERDAM   (Netherlands)

^b EMMA CHILDREN S HOSPITAL   (Netherlands)

^c UTRECHT UNIVERSITY   (Netherlands)

^d UNIVERSITY OF MÜNSTER   (Germany)

Author keywords

[No Author keywords available]

Indexed keywords

3 HYDROXYACYL COENZYME A DEHYDROGENASE; ACETYL COENZYME A ACETYLTRANSFERASE;

CONFERENCE PAPER; FATTY ACID OXIDATION; OXIDATION; PEROXISOME;

ACETYL-COA C-ACETYLTRANSFERASE; CARRIER PROTEINS; CHOLESTANOLS; FATTY ACIDS; HUMANS; MICROBODIES; OXIDATION-REDUCTION; PEROXISOMAL DISORDERS;

EID: 0031879234     PISSN: 01418955     EISSN: None     Source Type: Journal    
DOI: 10.1023/A:1005349028853     Document Type: Conference Paper

References (8)

1. Christensen E, Anken Pedersen S, Leth H, Jakobs C, Schutgens RBH, Wanders RJA (1997) A new peroxisomal β-oxidation disorder in twin neonates: defective oxidation of both cerotic and pristanic acids. J Inher Metab Dis 20: 658-664.
2. Jiang LL, Kurasawa T, Sao M, Suzuki Y, Hashimoto T (1997) Physiological role of D-3-hydroxyacyl-CoA dehydratase/D-3-hydroxyacyl-CoA dehydrogenase bifunctional protein. J Biochem 121: 506-513.
3. Miyazawa S, Osumi T, Hashimoto (1980) The presence of a new 3-oxoacyl-CoA thiolase in rat liver peroxisomes. Eur J Biochem 103: 589-596.
4. Seedorf F, Brysch P, Engel T et al (1994) Sterol carrier protein X is a peroxisomal 3-oxoacyl coenzyme A thiolase with intrinsic sterol carrier and lipid transfer activity. J Biol Chem 269: 21277-21283.
5. Verhoeven NM, Kulik W, van den Heuvel CMM, Jakobs C (1995) Pre- and postnatal diagnosis of peroxisomal disorders using stable-isotope dilution gas chromatography-mass spectrometry. J Inher Metab Dis 18 (supplement 1): 45-60.
6. Wanders RJA, Schutgens RBH, Barth PG (1995) Peroxisomal disorders: a review. J Neuropathol Exp Neurol 54: 726-739.
7. Wanders RJA, Denis S, Wouters F, Wirtz KWA, Seedorf U (1997) Sterol carrier protein X (SCPx) is a peroxisomal branched-chain β-ketothiolase specifically reacting with 3-oxopristanoyl-CoA: a new unique role for SCPx in branched-chain fatty acid metabolism in peroxisomes. Biochem Biophys Res Commun 236: 565-569.
8. Xu R, Cuebas DA (1996) The reactions catalyzed by the inducible bifunctional enzyme of rat liver peroxisomes cannot lead to the formation of bile acids. Biochem Biophys Res Commun 221: 271-278.