HOME HELP FEEDBACK SUBSCRIPTIONS ARCHIVE SEARCH TABLE OF CONTENTS		QUICK SEARCH:   [advanced] Author: Keyword(s): Year:  Vol:  Page:

This Article Full Text (PDF) Alert me when this article is cited Alert me if a correction is posted Citation Map Services Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Schrakamp, G. Articles by van den Bosch, H. Search for Related Content PubMed PubMed Citation Articles by Schrakamp, G. Articles by van den Bosch, H. Social Bookmarking                      What's this?

Journal of Lipid Research, Vol 26, 867-873, Copyright © 1985 by Lipid Research, Inc.

ARTICLES

Alkyl dihydroxyacetone phosphate synthase in human fibroblasts and its deficiency in Zellweger syndrome

G Schrakamp, CF Roosenboom, RB Schutgens, RJ Wanders, HS Heymans, JM Tager and H van den Bosch

The cerebro-hepato-renal (Zellweger) syndrome is an autosomal recessive disorder biochemically characterized by the absence of morphologically distinguishable peroxisomes. Key enzymes involved in the biosynthesis of ether phospholipids, i.e., dihydroxyacetone phosphate acyltransferase and alkyl dihydroxyacetone phosphate synthase, are located in mammalian (micro)peroxisomes. We have previously shown a strikingly reduced activity of dihydroxyacetone phosphate acyltransferase in liver, brain, and cultured skin fibroblasts from Zellweger patients (Schutgens et al. 1984. Biochim. Biophys. Res. Commun. 120: 179-184). We have now extended these investigations by studying alkyl dihydroxyacetone phosphate synthase in cultured human skin fibroblasts. Enzymatic activity was determined by measuring the formation of radioactive alkyl dihydroxyacetone phosphate from palmitoyl dihydroxyacetone phosphate and [1-14C]hexadecanol as substrates. The enzyme was optimally active at pH 8.5 and was stimulated (about 2-3-fold) by the presence of 0.05% (v/v) Triton X- 100. The apparent KM values for the enzyme in control fibroblasts amounted to 35 microM for palmitoyl dihydroxyacetone phosphate and 90 microM for hexadecanol. The reaction became inhibited at higher concentrations of both Triton X-100 and palmitoyl dihydroxyacetone phosphate. Control skin fibroblasts showed alkyl dihydroxyacetone phosphate synthase activity of 69 +/- 28 pmol X min-1 X mg-1 (n = 7), while fibroblasts from patients had an activity of only 6.3 +/- 1.7 pmol X min-1 X mg-1 (n = 7). Alkyl dihydroxyacetone phosphate synthase was also found to be deficient in tissue homogenates of Zellweger patients. The specific activity of this enzyme in liver, kidney, and brain homogenates from Zellweger patients was less than 15% of that in the corresponding tissues from controls.
CiteULike    Complore    Connotea    Del.icio.us    Digg    Reddit    Technorati    What's this?

This article has been cited by other articles:

				R. Zufferey, S. Allen, T. Barron, D. R. Sullivan, P. W. Denny, I. C. Almeida, D. F. Smith, S. J. Turco, M. A. J. Ferguson, and S. M. Beverley Ether Phospholipids and Glycosylinositolphospholipids Are Not Required for Amastigote Virulence or for Inhibition of Macrophage Activation by Leishmania major J. Biol. Chem., November 7, 2003; 278(45): 44708 - 44718. [Abstract] [Full Text] [PDF]

				T.-P. Thai, C. Rodemer, A. Jauch, A. Hunziker, A. Moser, K. Gorgas, and W. W. Just Impaired membrane traffic in defective ether lipid biosynthesis Hum. Mol. Genet., January 1, 2001; 10(2): 127 - 136. [Abstract] [Full Text] [PDF]

				S. Ferdinandusse, S. Denis, E. van Berkel, G. Dacremont, and R. J. A. Wanders Peroxisomal fatty acid oxidation disorders and 58 kDa sterol carrier protein X (SCPx): activity measurements in liver and fibroblasts using a newly developed method J. Lipid Res., March 1, 2000; 41(3): 336 - 342. [Abstract] [Full Text]

				E. C. J. M. de Vet, Y. H. A. Hilkes, M. W. Fraaije, and H. van den Bosch Alkyl-dihydroxyacetonephosphate Synthase. PRESENCE AND ROLE OF FLAVIN ADENINE DINUCLEOTIDE J. Biol. Chem., February 25, 2000; 275(9): 6276 - 6283. [Abstract] [Full Text] [PDF]

				E. C. J. M. de Vet, L. Ijlst, W. Oostheim, C. Dekker, H. W. Moser, H. van den Bosch, and R. J. A. Wanders Ether lipid biosynthesis: alkyl-dihydroxyacetonephosphate synthase protein deficiency leads to reduced dihydroxyacetonephosphate acyltransferase activities J. Lipid Res., November 1, 1999; 40(11): 1998 - 2003. [Abstract] [Full Text]

				M. Al-Essa, G. S. Dhaunsi, M. Rashed, P. T. Ozand, and Z. Rahbeeni Zeliweger Syndrome in Saudi Arabia and Its Distinct Features Clinical Pediatrics, March 1, 1999; 38(2): 77 - 86. [Abstract] [PDF]

				E. C. J. M. de Vet, L. IJlst, W. Oostheim, R. J. A. Wanders, and H. van den Bosch Alkyl-Dihydroxyacetonephosphate Synthase. FATE IN PEROXISOME BIOGENESIS DISORDERS AND IDENTIFICATION OF THE POINT MUTATION UNDERLYING A SINGLE ENZYME DEFICIENCY J. Biol. Chem., April 24, 1998; 273(17): 10296 - 10301. [Abstract] [Full Text] [PDF]