Characterization of mammalian sedoheptulokinase and mechanism of formation of erythritol in sedoheptulokinase deficiency

Tamas Kardon, Vincent Stroobant, Maria Veiga-da-Cunha, Emile Van Schaftingen

Research output: Contribution to journalArticle

14 Citations (Scopus)

Abstract

Our aim was to identify the product formed by sedoheptulokinase and to understand the mechanism of formation of erythritol in patients with sedoheptulokinase deficiency. Mouse recombinant sedoheptulokinase was found to be virtually specific for sedoheptulose and its reaction product was identified as sedoheptulose 7-phosphate. Assays of sedoheptulose in plant extracts disclosed that this sugar is present in carrots (≈7 μmol/g) and in several fruits. Sedoheptulose 1-phosphate is shown to be a substrate for aldolase B, which cleaves it to dihydroxyacetone-phosphate and erythrose. This suggests that, in patients deficient in sedoheptulose-7-kinase, sedoheptulose is phosphorylated by fructokinase to sedoheptulose 1-phosphate. Cleavage of the latter by aldolase B would lead to the formation of erythrose, which would then be reduced to erythritol.

Original languageEnglish
Pages (from-to)3330-3334
Number of pages5
JournalFEBS letters
Volume582
Issue number23-24
DOIs
Publication statusPublished - Oct 15 2008

Keywords

  • Cystinosis
  • Heptose
  • Pentose-phosphate pathway
  • Sedoheptulose
  • Sugar kinase
  • Volemitol

ASJC Scopus subject areas

  • Biophysics
  • Structural Biology
  • Biochemistry
  • Molecular Biology
  • Genetics
  • Cell Biology

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