FEBS Letters
Volume 582, Issue 23 , Pages 3330-3334, 15 October 2008

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

Edited by Judit Ovádi

  • Tamas Kardon

      Affiliations

    • Laboratory of Physiological Chemistry, de Duve Institute, Université Catholique de Louvain, Ave. Hippocrate 75, B-1200 Brussels, Belgium
    • ,
  • Vincent Stroobant

      Affiliations

    • The Ludwig Institute for Cancer Research, B-1200 Brussels, Belgium
    • ,
  • Maria Veiga-da-Cunha

      Affiliations

    • Laboratory of Physiological Chemistry, de Duve Institute, Université Catholique de Louvain, Ave. Hippocrate 75, B-1200 Brussels, Belgium
    • ,
  • Emile Van Schaftingen

      Affiliations

    • Laboratory of Physiological Chemistry, de Duve Institute, Université Catholique de Louvain, Ave. Hippocrate 75, B-1200 Brussels, Belgium
    • Corresponding Author InformationCorresponding author. Fax: +32 2 764 75 98.

Received 9 July 2008; received in revised form 6 August 2008; accepted 22 August 2008. published online 05 September 2008.

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 (≈7μmol/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.

Keywords: Sedoheptulose, Heptose, Cystinosis, Sugar kinase, Pentose-phosphate pathway, Volemitol

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PII: S0014-5793(08)00711-4

doi:10.1016/j.febslet.2008.08.024

FEBS Letters
Volume 582, Issue 23 , Pages 3330-3334, 15 October 2008

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