Involvement of microsomal fatty aldehyde dehydrogenase in the alpha-oxidation of phytanic acid

FEBS Letters
N M VerhoevenW B Rizzo

Abstract

We investigated the role of microsomal fatty aldehyde dehydrogenase (FALDH) in the conversion of pristanal into pristanic acid. Cultured skin fibroblasts from controls and patients with Sjögren-Larsson syndrome (SLS) who are genetically deficient in FALDH activity were incubated with [2,3-(3)H]phytanic acid. The release of aqueous-soluble radioactivity by the SLS cells was decreased to 25% of normal, consistent with an intact formation of pristanal but a deficiency of further oxidation. SLS cells also accumulated four-fold more radioactivity in N-alkyl-phosphatidyl ethanolamine, which arises from incorporation of free aldehyde into phosphatidyl ethanolamine. Recombinant human FALDH expressed in Chinese hamster ovary cells readily oxidized pristanal and cultured fibroblasts from SLS patients showed a severe deficiency in FALDH activity (13% of normal) when pristanal was used as substrate. Nevertheless, SLS patients did not accumulate phytanic acid in their plasma. We conclude that FALDH is involved in the oxidation of pristanal to pristanic acid and that this reaction is deficient in patients with SLS.

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Citations

Feb 13, 2001·Journal of the Neurological Sciences·M A WillemsenE Mayatepek
Oct 10, 2001·Progress in Lipid Research·N M Verhoeven, C Jakobs
Jun 20, 2003·Progress in Lipid Research·Mridul MukherjiMatthew D Lloyd
Apr 13, 2000·Biochimica Et Biophysica Acta·J K Hiltunen, Y Qin
Apr 16, 2003·European Journal of Biochemistry·Minne CasteelsPaul P Van Veldhoven
Jun 19, 2010·Journal of Lipid Research·Paul P Van Veldhoven
Jul 27, 2012·Journal of Inherited Metabolic Disease·Joris FuijkschotMichèl A A P Willemsen
Jan 27, 2004·Xenobiotica; the Fate of Foreign Compounds in Biological Systems·K UmeharaG Miyamoto
Aug 7, 2009·Molecular Genetics and Metabolism·P E SijensM Oudkerk
Aug 23, 2016·Expert Opinion on Orphan Drugs·William B Rizzo
Feb 19, 2008·The FEBS Journal·Matthew D LloydMichael D Threadgill
Jul 10, 2008·Expert Opinion on Drug Metabolism & Toxicology·Satori A MarchittiVasilis Vasiliou
Jun 23, 2011·Dermato-endocrinology·William B Rizzo
Aug 29, 2006·Biochimica Et Biophysica Acta·Gerbert A Jansen, Ronald J A Wanders
Oct 16, 2014·The Journal of Biological Chemistry·Ryo IwamaRyouichi Fukuda
Sep 13, 2019·Ophthalmic Genetics·Samiksha Fouzdar-JainWilliam B Rizzo
Oct 25, 2007·Biochemical Society Transactions·A S Wierzbicki
Apr 13, 1999·Biochemical and Biophysical Research Communications·S J SteinbergP A Watkins
Apr 18, 2001·Chemico-biological Interactions·A PappaV Vasiliou
Feb 5, 2000·The Journal of Pediatrics·M A WillemsenE Mayatepek
Jun 21, 2011·Biochimica Et Biophysica Acta·Ronald J A WandersSacha Ferdinandusse

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