Propionate enters GABAergic neurons, inhibits GABA transaminase, causes GABA accumulation and lethargy in a model of propionic acidemia

Dysregulated miRNAs and their pathogenic implications for the neurometabolic disease propionic acidemia

(2017) Ana Rivera-Barahona et al.   Scientific Reports

2-Methylcitric acid impairs glutamate metabolism and induces permeability transition in brain mitochondria

(2016) Alexandre Umpierrez Amaral et al.   JOURNAL OF NEUROCHEMISTRY

Uptake and metabolism of fructose by rat neocortical cellsin vivoand by isolated nerve terminalsin vitro

(2015) Bjørnar Hassel et al.   JOURNAL OF NEUROCHEMISTRY

Selected biomarkers as predictive tools in testing efficacy of melatonin and coenzyme Q on propionic acid - induced neurotoxicity in rodent model of autism

(2014) Mashael Al-Ghamdi et al.   BMC NEUROSCIENCE

High extracellular concentration of excitatory amino acids glutamate and aspartate in human brain abscess

(2014) Daniel Dahlberg et al.   NEUROCHEMISTRY INTERNATIONAL

Inhibition of N-acetylglutamate synthase by various monocarboxylic and dicarboxylic short-chain coenzyme A esters and the production of alternative glutamate esters

(2013) M. Dercksen et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE

Propionic acidemia: clinical course and outcome in 55 pediatric and adolescent patients

(2013) Sarah C Grünert et al.   Orphanet Journal of Rare Diseases

Patients with organic acidaemias have an altered thiol status

(2012) Heli Salmi et al.   ACTA PAEDIATRICA

Propionic Acidemia: Case Report and Review of Neurologic Sequelae

(2009) Jennifer A. Johnson et al.   PEDIATRIC NEUROLOGY

Striatal Medium-Sized Spiny Neurons: Identification by Nuclear Staining and Study of Neuronal Subpopulations in BAC Transgenic Mice

(2009) Miriam Matamales et al.   PLoS One