Familias GA | Wajner M.

P-199 Astrocytes pretreated with lysine or glutaric acid provoke neurotoxic effects in striatum and cerebral cortex from a Gcdh−/− mouse model 2014

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...

27 septiembre, 2022 / 0 Comentario

P-187 LYSINE AND GLUTARIC ACID AFFECTS CULTURED ASTROCYTES FROM THE KNOCKOUT MOUSE MODEL OF GLUTARIC ACIDEMIA TYPE I 2011

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...

27 septiembre, 2022 / 0 Comentario

P-183 LYSINE ADMINISTRATION PROMOTES OXIDATIVE STRESS IN BRAIN OF GLUTARYL-COA DEHYDROGENASE-DEFICIENT (GCDH−/−) MICE 2011

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...

27 septiembre, 2022 / 0 Comentario

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 2015

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 2015...

24 septiembre, 2022 / 0 Comentario

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 2012

Reduction of Na+, K+-ATPase activity and expression in cerebral cortex of glutaryl-CoA dehydrogenase defificient mice: A possible mechanism for brain injury in glutaric aciduria type I ...

24 septiembre, 2022 / 0 Comentario

418 – Striatum Histopathological Alterations Provoked by Acute Lysine Administration in Glutaryl-CoA Dehydrogenase Deficient Mice 2013

418 - Striatum Histopathological Alterations Provoked by Acute Lysine Administration in Glutaryl-CoA Dehydrogenase Deficient Mice 2013...

19 septiembre, 2022 / 0 Comentario

Elevated levels of BDNF and cathepsin‐d as possible peripheral markers of neurodegeneration in plasma of patients with glutaric acidemia type I 2019

Elevated levels of BDNF and cathepsin‐d as possible peripheral markers of neurodegeneration in plasma of patients with glutaric acidemia type I...

12 septiembre, 2020 / 0 Comentario

The role of oxidative damage in the neuropathology of organic acidurias: Insights from animal studies 2004

The role of oxidative damage in the neuropathology of organic acidurias: Insights from animal studies 2004 1wajner2004...

12 septiembre, 2020 / 0 Comentario

l-Carnitine prevents oxidative stress in striatum of glutaryl-CoA dehydrogenase deficient mice submitted to lysine overload 2019

l-Carnitine prevents oxidative stress in striatum of glutaryl-CoA dehydrogenase deficient mice submitted to lysine overload...

16 septiembre, 2019 / 0 Comentario

Selective Screening of Fatty Acids Oxidation Defects and Organic Acidemias 2018

Selective Screening of Fatty Acids Oxidation Defects and Organic Acidemias 2018...

02 agosto, 2019 / 0 Comentario

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