Microsomal fatty aldehyde dehydrogenase catalyzes the oxidation of aliphatic aldehyde derived from ether glycerolipid catabolism: Implications for Sjogren-Larsson syndrome

William B. Rizzo, Eric Heinz, Marcia Simon, Debra A. Craft

Research output: Contribution to journalArticle

36 Scopus citations

Abstract

The enzyme that catalyzes the oxidation of fatty aldehyde derived from ether glycerolipid catabolism has not been identified. To determine whether microsomal fatty aldehyde dehydrogenase (FALDH) is responsible, we investigated the metabolism of 1-O-[9,10-3 H-octadecyl]-glycerol ([3H]OG) in FALDH-deficient cultured cells from patients with Sjogren-Larsson syndrome (SLS) and in mutant Chinese hamster ovary (CHO) cells. Intact fibroblasts from SLS patients incubated with [3H]OG showed a selective deficiency (38±7% of normal) in the incorporation of radioactivity into fatty acid, but no decrease in incorporation of radioactivity into fatty alcohol, total lipids and phosphatidylethanolamine (PE). Consistent with fatty aldehyde accumulation, incorporation of radioactivity into N-alkyl-phosphatidylethanolamine, which is derived from Schiff base formation of free aldehyde with PE, was 4-fold higher in SLS fibroblasts compared to normal controls. Similar results were seen with SLS keratinocytes, whereas FALDH-deficient CHO cells showed a more profound reduction in radioactive fatty acid to 12±2% of normal. These results implicate FALDH in the oxidation of ether-derived fatty aldehyde in human and rodent cells. Metabolism of ether glycerolipids is a previously unrecognized source of fatty aldehyde that may contribute to the pathogenesis of SLS. (C) 2000 Elsevier Science B.V.

Original languageEnglish (US)
Pages (from-to)1-9
Number of pages9
JournalBiochimica et Biophysica Acta - Molecular Basis of Disease
Volume1535
Issue number1
DOIs
StatePublished - Dec 15 2000

Keywords

  • Aldehyde dehydrogenase
  • Ether lipid
  • Fatty aldehyde
  • Metabolism
  • Plasmalogen

ASJC Scopus subject areas

  • Molecular Medicine
  • Molecular Biology

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