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Eduard Alexander Struys

Metabolic Unit

Clinical Chemistry

VUMC Medical Center

De Boelelaan 1117



Name/email consistency: high



  • Metabolic Unit, Clinical Chemistry, VUMC Medical Center, De Boelelaan 1117, Netherlands. 2004 - 2012


  1. Pyridoxine-dependent Epilepsy With Elevated Urinary α-Amino Adipic Semialdehyde in Molybdenum Cofactor Deficiency. Struys, E.A., Nota, B., Bakkali, A., Al Shahwan, S., Salomons, G.S., Tabarki, B. Pediatrics (2012) [Pubmed]
  2. The measurement of urinary Δ¹-piperideine-6-carboxylate, the alter ego of α-aminoadipic semialdehyde, in Antiquitin deficiency. Struys, E.A., Bok, L.A., Emal, D., Houterman, S., Willemsen, M.A., Jakobs, C. J. Inherit. Metab. Dis. (2012) [Pubmed]
  3. Metabolism of lysine in alpha-aminoadipic semialdehyde dehydrogenase-deficient fibroblasts: evidence for an alternative pathway of pipecolic acid formation. Struys, E.A., Jakobs, C. FEBS Lett. (2010) [Pubmed]
  4. Novel insights into L-2-hydroxyglutaric aciduria: mass isotopomer studies reveal 2-oxoglutaric acid as the metabolic precursor of L-2-hydroxyglutaric acid. Struys, E.A., Gibson, K.M., Jakobs, C. J. Inherit. Metab. Dis. (2007) [Pubmed]
  5. D-2-hydroxyglutaric aciduria in three patients with proven SSADH deficiency: genetic coincidence or a related biochemical epiphenomenon?. Struys, E.A., Verhoeven, N.M., Salomons, G.S., Berthelot, J., Vianay-Saban, C., Chabrier, S., Thomas, J.A., Tsai, A.C., Gibson, K.M., Jakobs, C. Mol. Genet. Metab. (2006) [Pubmed]
  6. D-2-Hydroxyglutaric aciduria: unravelling the biochemical pathway and the genetic defect. Struys, E.A. J. Inherit. Metab. Dis. (2006) [Pubmed]
  7. Determination of the GABA analogue succinic semialdehyde in urine and cerebrospinal fluid by dinitrophenylhydrazine derivatization and liquid chromatography-tandem mass spectrometry: application to SSADH deficiency. Struys, E.A., Jansen, E.E., Gibson, K.M., Jakobs, C. J. Inherit. Metab. Dis. (2005) [Pubmed]
  8. Kinetic characterization of human hydroxyacid-oxoacid transhydrogenase: relevance to D-2-hydroxyglutaric and gamma-hydroxybutyric acidurias. Struys, E.A., Verhoeven, N.M., Ten Brink, H.J., Wickenhagen, W.V., Gibson, K.M., Jakobs, C. J. Inherit. Metab. Dis. (2005) [Pubmed]
  9. Investigations by mass isotopomer analysis of the formation of D-2-hydroxyglutarate by cultured lymphoblasts from two patients with D-2-hydroxyglutaric aciduria. Struys, E.A., Verhoeven, N.M., Brunengraber, H., Jakobs, C. FEBS Lett. (2004) [Pubmed]
  10. Measurement of urinary D- and L-2-hydroxyglutarate enantiomers by stable-isotope-dilution liquid chromatography-tandem mass spectrometry after derivatization with diacetyl-L-tartaric anhydride. Struys, E.A., Jansen, E.E., Verhoeven, N.M., Jakobs, C. Clin. Chem. (2004) [Pubmed]
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