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metabolic research group UCL
  Carbohydrate metabolism  
 
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  metabolic research group
   

Carbohydrate metabolism

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 Current research
 
Fructosamine metabolism
  Vitamin C biosynthesis
  Inborn errors of metabolism

 Publications
 Previous contributions
 

Purine nucleoside Analogues in Leukemia

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 Current research
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oxidative protein folding

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Fructosamine metabolism

Delplanque J, Delpierre G, Opperdoes FR, Van Schaftingen E. Tissue distribution and evolution of fructosamine 3-kinase and fructosamine 3-kinase-related protein. J Biol Chem 2004; 279:46606-13. pubmed

Fortpied J, Gemayel R, Stroobant V, Van Schaftingen E. Plant ribulosamine/erythrulosamine 3-kinase, a putative protein-repair enzyme. Biochem J 2005; 388:795-802.

Wiame E, Lamosa P, Santos H, Van Schaftingen E. Identification of glucoselysine-6-phosphate deglycase, an enzyme involved in the metabolism of the fructation product glucoselysine. Biochem J 2005; 392:263-9.

Delpierre G, Veiga-da-Cunha M, Vertommen D, Buysschaert M, Van Schaftingen E. Variability in erythrocyte fructosamine 3-kinase activity in humans correlates with polymorphisms in the FN3K gene and impacts on haemoglobin glycation at specific sites. Diabetes Metab 2006; 32:31-9.

Veiga da-Cunha M, Jacquemin P, Delpierre G, Godfraind C, Theate I, Vertommen D, Clotman F, Lemaigre F, Devuyst O, Van Schaftingen E. Increased protein glycation in fructosamine 3-kinase-deficient mice. Biochem J 2006 ; 399:257-64.

Fortpied J, Maliekal P, Vertommen D, Van Schaftingen E. Magnesium-dependent phosphatase-1 is a protein-fructosamine-6-phosphatase potentially involved in glycation repair. J Biol Chem 2006; 281:18378-85

Collard F, Vertommen D, Fortpied J, Duester G, Van Schaftingen E. Identification of 3-deoxyglucosone dehydrogenase as aldehyde dehydrogenase 1A1 (retinaldehyde dehydrogenase 1). Biochimie 2007; 89:369-73.

Fortpied J, Gemayel R, Vertommen D, Van Schaftingen E  Identification of protein-ribulosamine-5-phosphatase as human low-molecular-weight protein-tyrosine-phosphatase-A. Biochem J 2007 ; in press.

 

Vitamin C biosynthesis

Linster CL, Van Schaftingen E. Glucuronate, the precursor of vitamin C, is directly formed from UDP-glucuronate in liver. FEBS J (2006) 273:1516-27.

Linster CL, Van Schaftingen E. Vitamin C. Biosynthesis, recycling and degradation in mammals. FEBS J (2007) 274:1-22.

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Inborn errors of metabolism
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Veiga-da-Cunha M, Collet JF, Prieur B, Jaeken J, Peeraer Y, Rabbijns A, Van Schaftingen E. Mutations responsible for 3-phosphoserine phosphatase deficiency. Eur J Hum Genet 2004; 12:163-6.

Rzem R, Veiga-da-Cunha M, Noel G, Goffette S, Nassogne MC, Tabarki B, Scholler C, Marquardt T, Vikkula M, Van Schaftingen E. A gene encoding a putative FAD-dependent L-2-hydroxyglutarate dehydrogenase is mutated in L-2-hydroxyglutaric aciduria. Proc Natl Acad Sci USA 2004; 101:16849-54.

Struys EA, Salomons GS, Achouri Y, Van Schaftingen E, Grosso S, Craigen WJ, Verhoeven NM, Jakobs C. Mutations in the D-2-hydroxyglutarate dehydrogenase gene cause  D-2-hydroxyglutaric aciduria. Am. J Hum Genet 2005; 76:358-60

Maliekal P, Vertommen D, Delpierre G, Van Schaftingen E. Identification of the sequence encoding N-acetylneuraminate-9-phosphate phosphatase. Glycobiology 2006; 16:165-72.

Rzem R, Van Schaftingen E, Veiga-da-Cunha M. The gene mutated in l-2-hydroxyglutaric aciduria encodes l-2-hydroxyglutarate dehydrogenase. Biochimie 2006; 88:113-6.

Kardon T, Noel G, Vertommen D, Van Schaftingen E. Identification of the gene encoding hydroxyacid-oxoacid transhydrogenase, an enzyme that metabolizes 4-hydroxybutyrate. FEBS Lett 2006; 580:2347-50.

Achouri Y, Noel G, Van Schaftingen E. 2-Keto-4-methylthiobutyrate, an intermediate in the methionine salvage pathway, is a good substrate for CtBP1. Biochem Biophys Res Commun 2007; 352:903-6.

Hart CE, Race V, Achouri Y, Wiame E, Sharrard M, Olpin SE, Watkinson J, Bonham JR, Jaeken J, Matthijs G, Van Schaftingen E. Phosphoserine aminotransferase deficiency: a novel disorder of the serine biosynthesis pathway. Am J Hum Genet 2007; 80:931-7.

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