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. 1999 Jun;22(4):392-403.
doi: 10.1023/a:1005595921323.

Glutaric aciduria type I: pathomechanisms of neurodegeneration

K Ullrich  1 , B Flott-Rahmel, P Schluff, U Musshoff, A Das, T Lücke, R Steinfeld, E Christensen, C Jakobs, A Ludolph, A Neu, R Röper
Affiliations

Glutaric aciduria type I: pathomechanisms of neurodegeneration

K Ullrich et al. J Inherit Metab Dis. 1999 Jun.

Abstract

In organotypic corticostriatal and hippocampal slice cultures from rat brain, 3-hydroxyglutaric acid but not glutaric and glutaconic acids induced neurodegeneration by activation of NMDA receptors. Electrophysiological investigations (Xenopus laevis oocytes expressing glutamate receptors; rat mixed cortex culture) revealed no direct interaction of 3-hydroxyglutaric acid with glutamate receptors. We speculate that 3-hydroxyglutaric acid induces a mild energy deprivation that interferes with the voltage-dependent Mg(2+)-block of NMDA receptors.

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