096-P DYSMYELINATION IN EARLY-TREATED PATIENTS WITH GLUTARIC ACIDURIA TYPE I Lee NC1 , Chien YH1 , Peng SF1 , Cheng PW1 , Chang LM1 , Huang AC1,Hwu WL1 1 National Taiwan University Hospital, Taipei, Taiwan Background: Glutaric aciduria type I (GAI) is an inborn error of lysine and...
151-O Prediction and prevention of encephalopathy in a mouse model of GA1 2007 ...
Glutaric acidemia I (GAI) is a neurometabolic disease characterized by brain accumulation of glutaric acid (GA) and related metabolites and acute degeneration of striatal neurons through still unknown mechanisms. We investigated whether astrocytes from the Gcdh−/− mice submitted to lysine (Lys) or GA overload may...
Background: The role of glial cells in glutaric acidemia I (GA-I) pathogenesis is mostly unknown. Objectives: We evaluated the effects of pathophysiological concentrations of glutaric acid (GA) and lysine on glial cells from the glutaryl-CoA dehydrogenase (GCDH) knockout (KO) mouse model. Methods: Functional and morphological parameters were...
Background: The pathogenesis of the brain damage in glutaric acidemia type I (GA I) is not well understood. Objectives: Relevant oxidative stress parameters were investigated in cerebral cortex, striatum, hippocampus, liver and heart of 30-day-old knockout mice with glutaryl-CoA dehydrogenase deficiency (Gcdh −/−) and in...
418 - Striatum Histopathological Alterations Provoked by Acute Lysine Administration in Glutaryl-CoA Dehydrogenase Deficient Mice 2013...
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P-46. COMPARATIVA DEL CAMBIO DE DIETA EN 3 PACIENTES CON ACIDURIA GLUTÁRICA (AG) TIPO 1: CONTROL DE PROTEÍNA VS. CONTROL DE LISINA Egea Castillo N*1, de Los Santos de Pelegrín M1, García Arenas D1, Termes Escalé M1, Meavilla Olivas S1, Mínguez Rodríguez B1, García-Cazorla A2, Ormazabal...
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Lysine Glutarylation Is a Protein Post-Translational Modification 2014...