Eduard Alexander Struys
Metabolic Unit
Clinical Chemistry
VUMC Medical Center
De Boelelaan 1117
Netherlands
Name/email consistency: high
- 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)
- 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)
- 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)
- 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)
- 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)
- D-2-Hydroxyglutaric aciduria: unravelling the biochemical pathway and the genetic defect. Struys, E.A. J. Inherit. Metab. Dis. (2006)
- 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)
- 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)
- 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)
- 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)