Sort by
Items per page

Send to

Choose Destination

Search results

Items: 1 to 50 of 69


Inborn errors of metabolite repair.

Veiga-da-Cunha M, Van Schaftingen E, Bommer GT.

J Inherit Metab Dis. 2019 Nov 5. doi: 10.1002/jimd.12187. [Epub ahead of print] Review.


Metabolite Repair Enzymes Control Metabolic Damage in Glycolysis.

Bommer GT, Van Schaftingen E, Veiga-da-Cunha M.

Trends Biochem Sci. 2019 Aug 28. pii: S0968-0004(19)30176-8. doi: 10.1016/j.tibs.2019.07.004. [Epub ahead of print] Review.


The synthesis of branched-chain fatty acids is limited by enzymatic decarboxylation of ethyl- and methylmalonyl-CoA.

Dewulf JP, Gerin I, Rider MH, Veiga-da-Cunha M, Van Schaftingen E, Bommer GT.

Biochem J. 2019 Aug 30;476(16):2427-2447. doi: 10.1042/BCJ20190500.


Failure to eliminate a phosphorylated glucose analog leads to neutropenia in patients with G6PT and G6PC3 deficiency.

Veiga-da-Cunha M, Chevalier N, Stephenne X, Defour JP, Paczia N, Ferster A, Achouri Y, Dewulf JP, Linster CL, Bommer GT, Van Schaftingen E.

Proc Natl Acad Sci U S A. 2019 Jan 22;116(4):1241-1250. doi: 10.1073/pnas.1816143116. Epub 2019 Jan 9.


SETD3 protein is the actin-specific histidine N-methyltransferase.

Kwiatkowski S, Seliga AK, Vertommen D, Terreri M, Ishikawa T, Grabowska I, Tiebe M, Teleman AA, Jagielski AK, Veiga-da-Cunha M, Drozak J.

Elife. 2018 Dec 11;7. pii: e37921. doi: 10.7554/eLife.37921.


High-resolution mapping and recognition of lipid domains using AFM with toxin-derivatized probes.

Dumitru AC, Conrard L, Lo Giudice C, Henriet P, Veiga-da-Cunha M, Derclaye S, Tyteca D, Alsteens D.

Chem Commun (Camb). 2018 Jun 19;54(50):6903-6906. doi: 10.1039/c8cc02201a.


Nit1 is a metabolite repair enzyme that hydrolyzes deaminated glutathione.

Peracchi A, Veiga-da-Cunha M, Kuhara T, Ellens KW, Paczia N, Stroobant V, Seliga AK, Marlaire S, Jaisson S, Bommer GT, Sun J, Huebner K, Linster CL, Cooper AJL, Van Schaftingen E.

Proc Natl Acad Sci U S A. 2017 Apr 18;114(16):E3233-E3242. doi: 10.1073/pnas.1613736114. Epub 2017 Apr 3.


A conserved phosphatase destroys toxic glycolytic side products in mammals and yeast.

Collard F, Baldin F, Gerin I, Bolsée J, Noël G, Graff J, Veiga-da-Cunha M, Stroobant V, Vertommen D, Houddane A, Rider MH, Linster CL, Van Schaftingen E, Bommer GT.

Nat Chem Biol. 2016 Aug;12(8):601-7. doi: 10.1038/nchembio.2104. Epub 2016 Jun 13.


Carnosine and anserine homeostasis in skeletal muscle and heart is controlled by β-alanine transamination.

Blancquaert L, Baba SP, Kwiatkowski S, Stautemas J, Stegen S, Barbaresi S, Chung W, Boakye AA, Hoetker JD, Bhatnagar A, Delanghe J, Vanheel B, Veiga-da-Cunha M, Derave W, Everaert I.

J Physiol. 2016 Sep 1;594(17):4849-63. doi: 10.1113/JP272050. Epub 2016 Jun 2.


Cholesterol segregates into submicrometric domains at the living erythrocyte membrane: evidence and regulation.

Carquin M, Conrard L, Pollet H, Van Der Smissen P, Cominelli A, Veiga-da-Cunha M, Courtoy PJ, Tyteca D.

Cell Mol Life Sci. 2015 Dec;72(23):4633-51. doi: 10.1007/s00018-015-1951-x. Epub 2015 Jun 16.


A mouse model of L-2-hydroxyglutaric aciduria, a disorder of metabolite repair.

Rzem R, Achouri Y, Marbaix E, Schakman O, Wiame E, Marie S, Gailly P, Vincent MF, Veiga-da-Cunha M, Van Schaftingen E.

PLoS One. 2015 Mar 12;10(3):e0119540. doi: 10.1371/journal.pone.0119540. eCollection 2015.


Enzyme complexity in intermediary metabolism.

Van Schaftingen E, Veiga-da-Cunha M, Linster CL.

J Inherit Metab Dis. 2015 Jul;38(4):721-7. doi: 10.1007/s10545-015-9821-0. Epub 2015 Feb 21. Review.


Metabolite proofreading in carnosine and homocarnosine synthesis: molecular identification of PM20D2 as β-alanyl-lysine dipeptidase.

Veiga-da-Cunha M, Chevalier N, Stroobant V, Vertommen D, Van Schaftingen E.

J Biol Chem. 2014 Jul 11;289(28):19726-36. doi: 10.1074/jbc.M114.576579. Epub 2014 Jun 2.


Endogenous sphingomyelin segregates into submicrometric domains in the living erythrocyte membrane.

Carquin M, Pollet H, Veiga-da-Cunha M, Cominelli A, Van Der Smissen P, N'kuli F, Emonard H, Henriet P, Mizuno H, Courtoy PJ, Tyteca D.

J Lipid Res. 2014 Jul;55(7):1331-42. doi: 10.1194/jlr.M048538. Epub 2014 May 14.


Vertebrate Acyl CoA synthetase family member 4 (ACSF4-U26) is a β-alanine-activating enzyme homologous to bacterial non-ribosomal peptide synthetase.

Drozak J, Veiga-da-Cunha M, Kadziolka B, Van Schaftingen E.

FEBS J. 2014 Mar;281(6):1585-97. doi: 10.1111/febs.12725. Epub 2014 Feb 12.


C7orf10 encodes succinate-hydroxymethylglutarate CoA-transferase, the enzyme that converts glutarate to glutaryl-CoA.

Marlaire S, Van Schaftingen E, Veiga-da-Cunha M.

J Inherit Metab Dis. 2014 Jan;37(1):13-9. doi: 10.1007/s10545-013-9632-0. Epub 2013 Jul 27.


Metabolite proofreading, a neglected aspect of intermediary metabolism.

Van Schaftingen E, Rzem R, Marbaix A, Collard F, Veiga-da-Cunha M, Linster CL.

J Inherit Metab Dis. 2013 May;36(3):427-34. doi: 10.1007/s10545-012-9571-1. Epub 2013 Jan 8. Review.


Mutations in the AGXT2L2 gene cause phosphohydroxylysinuria.

Veiga-da-Cunha M, Verhoeven-Duif NM, de Koning TJ, Duran M, Dorland B, Van Schaftingen E.

J Inherit Metab Dis. 2013 Nov;36(6):961-6. doi: 10.1007/s10545-012-9568-9. Epub 2012 Dec 14.


Molecular identification of hydroxylysine kinase and of ammoniophospholyases acting on 5-phosphohydroxy-L-lysine and phosphoethanolamine.

Veiga-da-Cunha M, Hadi F, Balligand T, Stroobant V, Van Schaftingen E.

J Biol Chem. 2012 Mar 2;287(10):7246-55. doi: 10.1074/jbc.M111.323485. Epub 2012 Jan 12.


Ethylmalonyl-CoA decarboxylase, a new enzyme involved in metabolite proofreading.

Linster CL, Noël G, Stroobant V, Vertommen D, Vincent MF, Bommer GT, Veiga-da-Cunha M, Van Schaftingen E.

J Biol Chem. 2011 Dec 16;286(50):42992-3003. doi: 10.1074/jbc.M111.281527. Epub 2011 Oct 20.


Enzymatic repair of Amadori products.

Van Schaftingen E, Collard F, Wiame E, Veiga-da-Cunha M.

Amino Acids. 2012 Apr;42(4):1143-50. doi: 10.1007/s00726-010-0780-3. Epub 2010 Oct 22. Review.


Molecular identification of NAT8 as the enzyme that acetylates cysteine S-conjugates to mercapturic acids.

Veiga-da-Cunha M, Tyteca D, Stroobant V, Courtoy PJ, Opperdoes FR, Van Schaftingen E.

J Biol Chem. 2010 Jun 11;285(24):18888-98. doi: 10.1074/jbc.M110.110924. Epub 2010 Apr 13.


Molecular identification of carnosine synthase as ATP-grasp domain-containing protein 1 (ATPGD1).

Drozak J, Veiga-da-Cunha M, Vertommen D, Stroobant V, Van Schaftingen E.

J Biol Chem. 2010 Mar 26;285(13):9346-56. doi: 10.1074/jbc.M109.095505. Epub 2010 Jan 22.


Effects of fructosamine-3-kinase deficiency on function and survival of mouse pancreatic islets after prolonged culture in high glucose or ribose concentrations.

Pascal SM, Veiga-da-Cunha M, Gilon P, Van Schaftingen E, Jonas JC.

Am J Physiol Endocrinol Metab. 2010 Mar;298(3):E586-96. doi: 10.1152/ajpendo.00503.2009. Epub 2009 Dec 15.


Evolution of vertebrate glucokinase regulatory protein from a bacterial N-acetylmuramate 6-phosphate etherase.

Veiga-da-Cunha M, Sokolova T, Opperdoes F, Van Schaftingen E.

Biochem J. 2009 Oct 12;423(3):323-32. doi: 10.1042/BJ20090986.


Molecular identification of omega-amidase, the enzyme that is functionally coupled with glutamine transaminases, as the putative tumor suppressor Nit2.

Jaisson S, Veiga-da-Cunha M, Van Schaftingen E.

Biochimie. 2009 Sep;91(9):1066-71. doi: 10.1016/j.biochi.2009.07.002. Epub 2009 Jul 14.


L: -2-Hydroxyglutaric aciduria, a disorder of metabolite repair.

Van Schaftingen E, Rzem R, Veiga-da-Cunha M.

J Inherit Metab Dis. 2009 Apr;32(2):135-42. doi: 10.1007/s10545-008-1042-3. Epub 2008 Nov 21. Review.


Mammalian phosphomannomutase PMM1 is the brain IMP-sensitive glucose-1,6-bisphosphatase.

Veiga-da-Cunha M, Vleugels W, Maliekal P, Matthijs G, Van Schaftingen E.

J Biol Chem. 2008 Dec 5;283(49):33988-93. doi: 10.1074/jbc.M805224200. Epub 2008 Oct 16.


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

Kardon T, Stroobant V, Veiga-da-Cunha M, Schaftingen EV.

FEBS Lett. 2008 Oct 15;582(23-24):3330-4. doi: 10.1016/j.febslet.2008.08.024. Epub 2008 Sep 5.


Molecular identification of mammalian phosphopentomutase and glucose-1,6-bisphosphate synthase, two members of the alpha-D-phosphohexomutase family.

Maliekal P, Sokolova T, Vertommen D, Veiga-da-Cunha M, Van Schaftingen E.

J Biol Chem. 2007 Nov 2;282(44):31844-51. Epub 2007 Sep 5.


Many fructosamine 3-kinase homologues in bacteria are ribulosamine/erythrulosamine 3-kinases potentially involved in protein deglycation.

Gemayel R, Fortpied J, Rzem R, Vertommen D, Veiga-da-Cunha M, Van Schaftingen E.

FEBS J. 2007 Sep;274(17):4360-74. Epub 2007 Aug 6.


L-2-hydroxyglutaric aciduria, a defect of metabolite repair.

Rzem R, Vincent MF, Van Schaftingen E, Veiga-da-Cunha M.

J Inherit Metab Dis. 2007 Oct;30(5):681-9. Epub 2007 Jun 21.


Fructosamine 3-kinase and other enzymes involved in protein deglycation.

Van Schaftingen E, Delpierre G, Collard F, Fortpied J, Gemayel R, Wiame E, Veiga-da-Cunha M.

Adv Enzyme Regul. 2007;47:261-9. Epub 2007 Mar 6. Review. No abstract available.


Increased protein glycation in fructosamine 3-kinase-deficient mice.

Veiga da-Cunha M, Jacquemin P, Delpierre G, Godfraind C, Théate I, Vertommen D, Clotman F, Lemaigre F, Devuyst O, Van Schaftingen E.

Biochem J. 2006 Oct 15;399(2):257-64.


Variability in erythrocyte fructosamine 3-kinase activity in humans correlates with polymorphisms in the FN3K gene and impacts on haemoglobin glycation at specific sites.

Delpierre G, Veiga-da-Cunha M, Vertommen D, Buysschaert M, Van Schaftingen E.

Diabetes Metab. 2006 Feb;32(1):31-9.


The gene mutated in l-2-hydroxyglutaric aciduria encodes l-2-hydroxyglutarate dehydrogenase.

Rzem R, Van Schaftingen E, Veiga-da-Cunha M.

Biochimie. 2006 Jan;88(1):113-6. Epub 2005 Jun 23.


Fructose utilization in Lactococcus lactis as a model for low-GC gram-positive bacteria: its regulator, signal, and DNA-binding site.

Barrière C, Veiga-da-Cunha M, Pons N, Guédon E, van Hijum SA, Kok J, Kuipers OP, Ehrlich DS, Renault P.

J Bacteriol. 2005 Jun;187(11):3752-61.


Insights into the structure and regulation of glucokinase from a novel mutation (V62M), which causes maturity-onset diabetes of the young.

Gloyn AL, Odili S, Zelent D, Buettger C, Castleden HA, Steele AM, Stride A, Shiota C, Magnuson MA, Lorini R, d'Annunzio G, Stanley CA, Kwagh J, van Schaftingen E, Veiga-da-Cunha M, Barbetti F, Dunten P, Han Y, Grimsby J, Taub R, Ellard S, Hattersley AT, Matschinsky FM.

J Biol Chem. 2005 Apr 8;280(14):14105-13. Epub 2005 Jan 25.


New evidences for a regulation of deoxycytidine kinase activity by reversible phosphorylation.

Smal C, Bertrand L, Van den Neste E, Cardoen S, Veiga-da-Cunha M, Marie S, Race V, Ferrant A, Van den Berghe G, Bontemps F.

Nucleosides Nucleotides Nucleic Acids. 2004 Oct;23(8-9):1363-5.


A gene encoding a putative FAD-dependent L-2-hydroxyglutarate dehydrogenase is mutated in L-2-hydroxyglutaric aciduria.

Rzem R, Veiga-da-Cunha M, Noël G, Goffette S, Nassogne MC, Tabarki B, Schöller C, Marquardt T, Vikkula M, Van Schaftingen E.

Proc Natl Acad Sci U S A. 2004 Nov 30;101(48):16849-54. Epub 2004 Nov 17.


Identification of a dehydrogenase acting on D-2-hydroxyglutarate.

Achouri Y, Noël G, Vertommen D, Rider MH, Veiga-Da-Cunha M, Van Schaftingen E.

Biochem J. 2004 Jul 1;381(Pt 1):35-42.


Mutations responsible for 3-phosphoserine phosphatase deficiency.

Veiga-da-Cunha M, Collet JF, Prieur B, Jaeken J, Peeraer Y, Rabbijns A, Van Schaftingen E.

Eur J Hum Genet. 2004 Feb;12(2):163-6.


Mutations in the glucokinase regulatory protein gene in 2p23 in obese French caucasians.

Veiga-da-Cunha M, Delplanque J, Gillain A, Bonthron DT, Boutin P, Van Schaftingen E, Froguel P.

Diabetologia. 2003 May;46(5):704-11. Epub 2003 May 9.


Identification of fructose 6-phosphate- and fructose 1-phosphate-binding residues in the regulatory protein of glucokinase.

Veiga-da-Cunha M, Van Schaftingen E.

J Biol Chem. 2002 Mar 8;277(10):8466-73. Epub 2001 Dec 27.


Glucose-6-phosphatase mutation G188R confers an atypical glycogen storage disease type 1b phenotype.

Weston BW, Lin JL, Muenzer J, Cameron HS, Arnold RR, Seydewitz HH, Mayatepek E, Van Schaftingen E, Veiga-da-Cunha M, Matern D, Chen YT.

Pediatr Res. 2000 Sep;48(3):329-34.


How many forms of glycogen storage disease type I?

Veiga-da-Cunha M, Gerin I, Van Schaftingen E.

Eur J Pediatr. 2000 May;159(5):314-8. Review.


Overexpression and purification of fructose-1-phosphate kinase from Escherichia coli: application to the assay of fructose 1-phosphate.

Veiga-da-Cunha M, Hoyoux A, Van Schaftingen E.

Protein Expr Purif. 2000 Jun;19(1):48-52. Erratum in: Protein Expr Purif 2000 Oct;20(1):132. Houyoux, A [corrected to Hoyoux, A].


Identification of the cDNA encoding human 6-phosphogluconolactonase, the enzyme catalyzing the second step of the pentose phosphate pathway(1).

Collard F, Collet JF, Gerin I, Veiga-da-Cunha M, Van Schaftingen E.

FEBS Lett. 1999 Oct 8;459(2):223-6.


The putative glucose 6-phosphate translocase gene is mutated in essentially all cases of glycogen storage disease type I non-a.

Veiga-da-Cunha M, Gerin I, Chen YT, Lee PJ, Leonard JV, Maire I, Wendel U, Vikkula M, Van Schaftingen E.

Eur J Hum Genet. 1999 Sep;7(6):717-23.

Supplemental Content

Loading ...
Support Center