Review Pathogenesis of brain damage in glutaric acidemia type I: Lessons from the genetic mice model 2019
Pathogenesis of brain damage in glutaric acidemia type I Lessons from the genetic mice model 2019...
Asociación sin ánimo de lucro, inscrita en el Registro Nacional de Asociaciones con el número 613.620. Pertenece a FEDER, Federación Española de Enfermedades Raras que une a toda la comunidad de familias con enfermedades poco frecuentes en España; y a FEEHM, Federación Española de Enfermedades Metabólicas Hereditarias.
Pathogenesis of brain damage in glutaric acidemia type I Lessons from the genetic mice model 2019...
Increased susceptibility to quinolinic acid-induced seizures and long term changes in brain oscillations in an animal model of glutaric acidemia type I 2021...
Expanded Newborn Screening. Six-year experience in a single centre 2018 pag 76...
Expanded Newborn Screening. Six-year experience in a single centre 2018 pag 76...
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...
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...
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 ...
418 - Striatum Histopathological Alterations Provoked by Acute Lysine Administration in Glutaryl-CoA Dehydrogenase Deficient Mice 2013...
Este sitio web utiliza cookies para que usted tenga la mejor experiencia de usuario. Si continúa navegando está dando su consentimiento para la aceptación de las mencionadas cookies y la aceptación de nuestra política de cookies, pinche el enlace para mayor información.
ACEPTAR