title: Astrocyte Dysfunction in Developmental Neurometabolic Diseases.
abstract: Astrocytes play crucial roles in maintaining brain homeostasis and in orchestrating neural development, all through tightly coordinated steps that cooperate to maintain the balance needed for normal development. Here, we review the alterations in astrocyte functions that contribute to a variety of developmental neurometabolic disorders and provide additional data on the predominant role of astrocyte dysfunction in the neurometabolic neurodegenerative disease glutaric acidemia type I. Finally, we describe some of the therapeutical approaches directed to neurometabolic diseases and discuss if astrocytes can be possible therapeutic targets for treating these disorders.
authors.affiliation: Cellular and Molecular Neurobiology, Instituto de Investigaciones Biológicas Clemente Estable (IIBCE), Montevideo, Uruguay. solivera@iibce.edu.uy.
authors.forename: Silvia
authors.initials: S
authors.lastname: Olivera-Bravo
authors.affiliation: Cellular and Molecular Neurobiology, Instituto de Investigaciones Biológicas Clemente Estable (IIBCE), Montevideo, Uruguay.
authors.forename: Eugenia
authors.initials: E
authors.lastname: Isasi
authors.affiliation: Neuroscience Division, IIBCE and Comparative Neuroanatomy-Associated Unit to the School of Sciences, UDELAR, Montevideo, Uruguay.
authors.forename: Anabel
authors.initials: A
authors.lastname: Fernández
authors.affiliation: UMET, School of Sciences, UDELAR, Montevideo, Uruguay.
authors.forename: Gabriela
authors.initials: G
authors.lastname: Casanova
authors.affiliation: Neuroscience Division IIBCE, UDELAR, Montevideo, Uruguay.
authors.forename: Juan Carlos
authors.initials: JC
authors.lastname: Rosillo
authors.affiliation: Neurodegeneration Laboratory, Institut Pasteur Montevideo, Montevideo, Uruguay.
authors.forename: Luigi
authors.initials: L
authors.lastname: Barbeito
mesh_terms: D038261:Alexander Disease / Q000175:diagnosis / Q000378:metabolism / Q000473:pathology / Q000628:therapy; D000592:Amino Acid Metabolism, Inborn Errors / Q000175:diagnosis* / Q000378:metabolism / Q000473:pathology / Q000628:therapy*; D000975:Antioxidants / Q000627:therapeutic use; D001253:Astrocytes / Q000187:drug effects / Q000378:metabolism / Q000473:pathology*; D001921:Brain / Q000187:drug effects / Q000378:metabolism / Q000473:pathology*; D001928:Brain Diseases, Metabolic / Q000175:diagnosis* / Q000378:metabolism / Q000473:pathology / Q000628:therapy*; D002570:Ceruloplasmin / Q000172:deficiency / Q000378:metabolism; D004032:Diet / Q000379:methods; D019468:Disease Management; D005947:Glucose / Q000627:therapeutic use; D005974:Glutamate-Ammonia Ligase / Q000172:deficiency / Q000378:metabolism; D050770:Glutaryl-CoA Dehydrogenase / Q000172:deficiency* / Q000378:metabolism; D006501:Hepatic Encephalopathy / Q000175:diagnosis / Q000378:metabolism / Q000473:pathology / Q000628:therapy; D006706:Homeostasis; D006801:Humans; D019189:Iron Metabolism Disorders / Q000175:diagnosis / Q000378:metabolism / Q000473:pathology / Q000628:therapy; D019636:Neurodegenerative Diseases / Q000175:diagnosis / Q000378:metabolism / Q000473:pathology / Q000628:therapy; D055495:Neurogenesis / Q000187:drug effects; D052556:Niemann-Pick Disease, Type C / Q000175:diagnosis / Q000378:metabolism / Q000473:pathology / Q000628:therapy; D015324:Pyruvate Carboxylase Deficiency Disease / Q000175:diagnosis / Q000378:metabolism / Q000473:pathology / Q000628:therapy; D016060:Sorption Detoxification
chemical_list: D000975:Antioxidants; D002570:Ceruloplasmin; D050770:Glutaryl-CoA Dehydrogenase; D005974:Glutamate-Ammonia Ligase; D005947:Glucose
keywords: Astrocyte dysfunction; Astrocytes; Myelin damage; Neurodegeneration; Neurodevelopmental diseases