Keywords: نوع I اسید گلوتاریک; Glutaric acidemia type I; Glutaric acid; Gcdhâ/â mice; Citric acid cycle; Bioenergetics homeostasis; Brain histopathology;
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Selective and accurate C5 acylcarnitine quantitation by UHPLC-MS/MS: Distinguishing true isovaleric acidemia from pivalate derived interference
Keywords: نوع I اسید گلوتاریک; IVA; isovaleric acidemia; SCADD; short chain acyl-CoA dehydrogenase deficiency; GAI; glutaric acidemia type I; SCX; strong cation exchange; SPE; solid-phase extraction; LLOQ; lower limit of quantitation; ULOQ; upper limit of quantitation; CAP; College of
Toxic synergism between quinolinic acid and organic acids accumulating in glutaric acidemia type I and in disorders of propionate metabolism in rat brain synaptosomes: Relevance for metabolic acidemias
Keywords: نوع I اسید گلوتاریک; GA; glutaric acid; GA I; glutaric acidemia type I; GDD; glutaryl-CoA dehydrogenase; HEPES; 4-(2-hydroxyethyl)-1-piperazineethanesulfonic acid; KA; kynurenic acid; KP; kynurenine pathway; l-NAME; l-nitro-l-arginine methyl ester; MDA; Malondialdehyde; MMA;
Acute lysine overload provokes protein oxidative damage and reduction of antioxidant defenses in the brain of infant glutaryl-CoA dehydrogenase deficient mice: A role for oxidative stress in GA I neuropathology
Keywords: نوع I اسید گلوتاریک; Glutaric acidemia type I; Brain damage; Antioxidant defenses; Glutaric acid; 3-Hydroxyglutaric acid; Infant Gcdh−/− mice
Neurodevelopmental and cognitive behavior of glutaryl-CoA dehydrogenase deficient knockout mice
Keywords: نوع I اسید گلوتاریک; Glutaric acidemia type I; Mice behavior; Cognitive behavior; Climbing behavior; Neurodevelopment; Knockout mice model;
Marked reduction of Na+, K+-ATPase and creatine kinase activities induced by acute lysine administration in glutaryl-CoA dehydrogenase deficient mice
Keywords: نوع I اسید گلوتاریک; CK; creatine kinase; DCIP; dichloroindophenol; EDTA; ethylenediaminetetracetic acid; GA; glutaric acid; GA I; glutaric acidemia type I; GCDH; glutaryl-CoA dehydrogenase; Gcdhâ/â; glutaryl-CoA dehydrogenase deficient mice; 3HGA; 3-hydroxyglutaric acid;
Reduction of Na+, K+-ATPase activity and expression in cerebral cortex of glutaryl-CoA dehydrogenase deficient mice: A possible mechanism for brain injury in glutaric aciduria type I
Keywords: نوع I اسید گلوتاریک; CK; creatine kinase; DCIP; dichloroindophenol; DNP; dinitrophenol; EDTA; ethylenediaminetetracetic acid; EGTA; ethylene glycol-bis(2-aminoethylether)-N,N,Nâ²,Nâ²-tetraacetic acid; GA; glutaric acid; GA I; glutaric acidemia type I; GCDH; glutaryl-CoA deh
Chronic early postnatal glutaric acid administration causes cognitive deficits in the water maze
Keywords: نوع I اسید گلوتاریک; Glutaric acidemia type I; Glutaric acid; Memory; Water maze; Rat
L'acidurie glutarique type I : une cause méconnue de dystonie progressive
Keywords: نوع I اسید گلوتاریک; Acidurie Glutarique type I; Dystonie; Anomalies IRM; Variabilité phénotypique; Glutaric acidemia type I; Dystonia; MRI abnormalities; Phenotypic variability;
Newborn screening for metabolic disorders
Keywords: نوع I اسید گلوتاریک; FAOD; Fatty acid oxidation disorders; GA-I; Glutaric acidemia type I; GALT; Galactose-1-phosphate uridyltransferase; IVA; Isovaleric acidemia; MCADD; Medium chain acyl-CoA dehydrogenase deficiency; 3-MCCD; 3-Methylcrotonyl-CoA carboxylase deficiency; MS/M
Glutaric acid moderately compromises energy metabolism in rat brain
Keywords: نوع I اسید گلوتاریک; Glutaric acid; Glutaric acidemia type I; Creatine kinase inhibition; Respiratory chain complex inhibition; Mitochondrial respiratory parameters;
The urinary excretion of glutarylcarnitine is an informative tool in the biochemical diagnosis of glutaric acidemia type I
Keywords: نوع I اسید گلوتاریک; Glutaric acidemia type I; Organic aciduria; Acylcarnitine analysis; Organic acid analysis; Biochemical genetics;