|1.||Pons, R: 1 article (01/2015)|
|2.||Dinopoulos, A: 1 article (01/2015)|
|3.||Liadinioti, C: 1 article (01/2015)|
|4.||Stefanis, L: 1 article (01/2015)|
|5.||Fraidakis, M J: 1 article (01/2015)|
|6.||Ribes, A: 1 article (01/2015)|
|7.||Garcia-Villoria, J: 1 article (01/2015)|
|8.||Papathanassiou, M: 1 article (01/2015)|
|9.||Koeller, David M: 1 article (05/2011)|
|10.||Ullrich, Kurt: 1 article (05/2011)|
01/01/2015 - "At a presymptomatic stage diagnosis is suggested clinically by macrocephaly, radiologically by widened Sylvian fissures and biochemically by the presence of excess 3-hydroxyglutaric acid and glutaric acid in urine. "
06/01/2005 - "In the work up for macrocephaly urinary organic acids were determined showing a glutaric acid: 78,000 mmol/mol creatinine (normal values: 2-10); 3-hydroxyglutaric acid: 250 mmol/mol creatinine (normal values: 1-12). "
|2.||Neurodegenerative Diseases (Neurodegenerative Disease)
05/20/2011 - "The inherited neurodegenerative disorder glutaric aciduria type 1 (GA1) results from mutations in the gene for the mitochondrial matrix enzyme glutaryl-CoA dehydrogenase (GCDH), which leads to elevations of the dicarboxylates glutaric acid (GA) and 3-hydroxyglutaric acid (3OHGA) in brain and blood. "
10/19/2001 - "We conclude that 3-hydroxyglutaric acid induces convulsions and striatal damage via initiation of an imbalance in the excitatory glutamatergic and the inhibitory GABAergic neurotransmission, resulting in an enhanced excitatory input in striatal neurons. "
10/19/2001 - "Here, we report that 3-hydroxyglutaric acid induced an increase in convulsion frequency and duration as determined by open field measurement. "
10/19/2001 - "Intrastriatal administration of 3-hydroxyglutaric acid induces convulsions and striatal lesions in rats."
10/19/2001 - "N-methyl-D-aspartate (NMDA) receptor blockade by MK-801 and stimulation of GABA(A) receptors by muscimol prevented the induction of convulsions and striatal damage by 3-hydroxyglutaric acid, whereas blockade of non-NMDA receptors by 6,7-dinitroquinoxaline-2,3-dione (DNQX) was not protective. "
|4.||Dizocilpine Maleate (Dizocilpine)
|6.||N-Methyl-D-Aspartate Receptors (NMDA Receptors)
|7.||GABA-A Receptors (GABA(A) Receptor)