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

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Kardon, Tamas [UCL] Stroobant, Vincent [UCL] Veiga da Cunha, Maria [UCL] Van Schaftingen, Emile [UCL]

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 ( approximately 7mumol/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.

Document type Article de périodique (Journal article) – Article de recherche – Journal Article, Research Support, Non-U.S. Gov't
Access type Accès restreint
Publication date 2008
Language Anglais
Journal information "FEBS Letters" - Vol. 582, no. 23-24, p. 3330-3334 (2008)
Peer reviewed yes
Publisher Elsevier BV ((Netherlands) Amsterdam)
issn 0014-5793
e-issn 1873-3468
Publication status Publié
Affiliation UCL - MD/BICL - Département de biochimie et de biologie cellulaire
MESH Subject Vegetables - enzymology ; Transcription Factors - chemistry - deficiency - genetics ; Sugar Phosphates - chemistry - metabolism ; Recombinant Proteins - chemistry - genetics - metabolism ; Plant Proteins - chemistry - genetics - metabolism ; Phosphotransferases (Alcohol Group Acceptor) - chemistry - deficiency - genetics - metabolism ; Mice ; Humans ; Fruit - enzymology ; Fructose-Bisphosphate Aldolase - chemistry ; Erythritol - biosynthesis ; Animals
Keywords Sedoheptulose ; Heptose ; Cystinosis ; Sugar kinase ; Pentose-phosphate pathway ; Volemitol
Links
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Bibliographic reference Kardon, Tamas ; Stroobant, Vincent ; Veiga da Cunha, Maria ; Van Schaftingen, Emile. Characterization of mammalian sedoheptulokinase and mechanism of formation of erythritol in sedoheptulokinase deficiency. In: FEBS Letters, Vol. 582, no. 23-24, p. 3330-3334 (2008)
Permanent URL http://hdl.handle.net/2078.1/19564