Glutaric aciduria type I: pathomechanisms of neurodegeneration

B. Flott-Rahmel, P. Schluff, U. Musshoff, E.A. Das-Smaal, T. Lucke, R. Steinfeld, E. Christensen, C.A.J.M. Jakobs, A. Ludolph, A. Neu, R. Roper, K. Ullrich

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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
Original languageEnglish
Pages (from-to)392-403
JournalJournal of Inherited Metabolic Disease
Publication statusPublished - 1999


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