Ammonium accumulation and chemokine decrease in culture media of Gcdh

Autor:	Hong-Phuc, Cudré-Cung, Noémie, Remacle, Sonia, do Vale-Pereira, Mary, Gonzalez, Hugues, Henry, Julijana, Ivanisevic, Jessica, Schmiesing, Chris, Mühlhausen, Olivier, Braissant, Diana, Ballhausen
Rok vydání:	2018
Předmět:	Mice Knockout Glutaryl-CoA Dehydrogenase Tissue Scaffolds Brain Diseases Metabolic Lysine Cell Culture Techniques Brain Culture Media Mice Inbred C57BL Mice Ammonium Compounds Animals Chemokines Amino Acid Metabolism Inborn Errors
Zdroj:	Molecular genetics and metabolism. 126(4)
ISSN:	1096-7206
Popis:	Glutaric Aciduria type I (GA-I) is caused by mutations in the GCDH gene. Its deficiency results in accumulation of the key metabolites glutaric acid (GA) and 3-hydroxyglutaric acid (3-OHGA) in body tissues and fluids. Present knowledge on the neuropathogenesis of GA-I suggests that GA and 3-OHGA have toxic properties on the developing brain. We analyzed morphological and biochemical features of 3D brain cell aggregates issued from Gcdh
Databáze:	OpenAIRE
Externí odkaz:	https://explore.openaire.eu/search/publication?articleId=pmid________::509b461b5f69a402488c69cafec7a481 https://pubmed.ncbi.nlm.nih.gov/30686684 Zobrazit plný text záznamu Full Text from ScienceDirect