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J Inherit Metab Dis
1999 Jun 01;224:392-403. doi: 10.1023/a:1005595921323.
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Glutaric aciduria type I: pathomechanisms of neurodegeneration.
Ullrich K
,
Flott-Rahmel B
,
Schluff P
,
Musshoff U
,
Das A
,
Lücke T
,
Steinfeld R
,
Christensen E
,
Jakobs C
,
Ludolph A
,
Neu A
,
Röper R
.
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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(2+)-block of NMDA receptors.
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