Article ID | Journal | Published Year | Pages | File Type |
---|---|---|---|---|
10833799 | Molecular Genetics and Metabolism | 2011 | 13 Pages |
Abstract
⺠Despite numerous studies, neuropathogenesis of GA-I is only partially understood. ⺠Animal models and in vitro studies focusing on neurological damage are summarized. ⺠Intra-neuronal accumulation of GA seems to be an important pathomechanism. ⺠Excitotoxicity, impairment of energy metabolism and oxidative stress are involved. ⺠Intracerebral trapping of GA metabolites contribute to neuronal damage.
Keywords
RNSTCAGSHGPXBCBs.c.i.v.iNOSDCFH-DAi.p.NR2BDNQXAMPATRAPAPVIL-1βIFN-γN-methyl-d-aspartateNMDAGCDHSDHglutaric aciduria type INMGGA-IPDHCATN-methyl-d-aspartate receptor subunit 2BPDC2′,7′-dichlorofluorescein diacetateMAPKROS3-hydroxyglutaric acidα-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acidQuinolinic acidglutaric acidOctexcitotoxicityinterferon-γInterleukin-1βChoTarChinese Hamster OvaryOxidative stressOatorganic cation transporterorganic anion transporterintra peritonealintra venousCNSSODBlood–brain barrierBBBinducible nitric oxide synthaseSuperoxide dismutasesuccinate dehydrogenasecentral nervous systemEnergy failureVascular endothelial growth factorVascular Endothelial Growth Factor (VEGF)Phosphocreatinenot applicableBlood–cerebrospinal fluid barrierNeuropathogenesisNitric oxidePCRmitogen-activated protein kinasepyruvate dehydrogenasetricarboxylic acid cycleCatalasecreatineCreatine kinaseGluGlutathioneglutathione peroxidaseglutaryl-CoA dehydrogenaseglutamatereactive nitrogen speciesReactive oxygen species
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Authors
Paris Jafari, Olivier Braissant, Luisa Bonafé, Diana Ballhausen,