Neuropathological, biochemical and molecular findings in a glutaric acidemia type 1 cohort.
about
Natural history, outcome, and treatment efficacy in children and adults with glutaryl-CoA dehydrogenase deficiencyRare Late-Onset Presentation of Glutaric Aciduria Type I in a 16-Year-Old Woman with a Novel GCDH Mutation.Glutaric acid-mediated apoptosis in primary striatal neuronsNeonatal astrocyte damage is sufficient to trigger progressive striatal degeneration in a rat model of glutaric acidemia-I.Increased blood-brain barrier permeability and alterations in perivascular astrocytes and pericytes induced by intracisternal glutaric acid.Cerebral haemodynamics in patients with glutaryl-coenzyme A dehydrogenase deficiency.Astrocyte Dysfunction in Developmental Neurometabolic Diseases.Effects of targeted suppression of glutaryl-CoA dehydrogenase by lentivirus-mediated shRNA and excessive intake of lysine on apoptosis in rat striatal neurons.N-acetylcysteine prevents spatial memory impairment induced by chronic early postnatal glutaric acid and lipopolysaccharide in rat pups.Increased glutamate receptor and transporter expression in the cerebral cortex and striatum of gcdh-/- mice: possible implications for the neuropathology of glutaric acidemia type I.Mechanism of age-dependent susceptibility and novel treatment strategy in glutaric acidemia type IDiagnosis and Genetic Analysis of Glutaric Acidaemia Type I: Very rarely seen inborn error of metabolismA cross-sectional controlled developmental study of neuropsychological functions in patients with glutaric aciduria type I.Glutaric acidemia type 1Diagnosis, treatment and outcome of glutaric aciduria type I in Zhejiang Province, China.Neural transplants in patients with Huntington's disease undergo disease-like neuronal degeneration.Brain MRI findings as an important diagnostic clue in glutaric aciduria type 1.The mechanism of degeneration of striatal neuronal subtypes in Huntington diseaseA role of astrocytes in mediating postnatal neurodegeneration in Glutaric acidemia-type 1.Higher Vulnerability of Menadione-Exposed Cortical Astrocytes of Glutaryl-CoA Dehydrogenase Deficient Mice to Oxidative Stress, Mitochondrial Dysfunction, and Cell Death: Implications for the Neurodegeneration in Glutaric Aciduria Type I.The child with glutaric aciduria type I: anesthetic and perioperative management.Excessive homozygosity identified by chromosomal microarray at a known GCDH mutation locus correlates with brain MRI abnormalities in an infant with glutaric aciduria.Agenesis of the corpus callosum and cerebral anomalies in inborn errors of metabolism.The sodium-dependent di- and tricarboxylate transporter, NaCT, is not responsible for the uptake of D-, L-2-hydroxyglutarate and 3-hydroxyglutarate into neuronsWhite matter injury induced by perinatal exposure to glutaric acid.Endothelial effects of 3-hydroxyglutaric acid: implications for glutaric aciduria type I.Dynamic changes of striatal and extrastriatal abnormalities in glutaric aciduria type I.α-Ketoadipic Acid and α-Aminoadipic Acid Cause Disturbance of Glutamatergic Neurotransmission and Induction of Oxidative Stress In Vitro in Brain of Adolescent Rats.Exposure to intrauterine inflammation alters metabolomic profiles in the amniotic fluid, fetal and neonatal brain in the mouse.Induction of Neuroinflammatory Response and Histopathological Alterations Caused by Quinolinic Acid Administration in the Striatum of Glutaryl-CoA Dehydrogenase Deficient Mice.Impairment of astrocytic glutaminolysis in glutaric aciduria type I.Multimodal imaging of striatal degeneration in Amish patients with glutaryl-CoA dehydrogenase deficiency.Striatal neuronal death mediated by astrocytes from the Gcdh-/- mouse model of glutaric acidemia type I.(1)H-MRS in glutaric aciduria type 1: impact of biochemical phenotype and age on the cerebral accumulation of neurotoxic metabolites.m-Trifluoromethyl diphenyl diselenide attenuates glutaric acid-induced seizures and oxidative stress in rat pups: involvement of the γ-aminobutyric acidergic system.Unravelling the complex MRI pattern in glutaric aciduria type I using statistical models-a cohort study in 180 patients.Anesthetic Management for Fracture Head of Radius in a Child with Glutaric Aciduria type-1.
P2860
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P2860
Neuropathological, biochemical and molecular findings in a glutaric acidemia type 1 cohort.
description
2005 nî lūn-bûn
@nan
2005年の論文
@ja
2005年論文
@yue
2005年論文
@zh-hant
2005年論文
@zh-hk
2005年論文
@zh-mo
2005年論文
@zh-tw
2005年论文
@wuu
2005年论文
@zh
2005年论文
@zh-cn
name
Neuropathological, biochemical ...... utaric acidemia type 1 cohort.
@en
Neuropathological, biochemical ...... utaric acidemia type 1 cohort.
@nl
type
label
Neuropathological, biochemical ...... utaric acidemia type 1 cohort.
@en
Neuropathological, biochemical ...... utaric acidemia type 1 cohort.
@nl
prefLabel
Neuropathological, biochemical ...... utaric acidemia type 1 cohort.
@en
Neuropathological, biochemical ...... utaric acidemia type 1 cohort.
@nl
P2093
P356
P1433
P1476
Neuropathological, biochemical ...... utaric acidemia type 1 cohort.
@en
P2093
Asuri N Prasad
Cheryl R Greenberg
Christopher B R Funk
Stefan Kölker
Sven Sauer
P304
P356
10.1093/BRAIN/AWH401
P407
P577
2005-02-02T00:00:00Z