Astrocyte Dysfunction in Developmental Neurometabolic Diseases.

Silvia Olivera-Bravo, Eugenia Isasi, Anabel Fernández, Gabriela Casanova, Juan Carlos Rosillo, Luigi Barbeito

Journal: Advances in experimental medicine and biology 2017;949():227-243

PMID: 27714692

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.

Address: Cellular and Molecular Neurobiology, Instituto de Investigaciones Biológicas Clemente Estable (IIBCE), Montevideo, Uruguay. [email protected].; Cellular and Molecular Neurobiology, Instituto de Investigaciones Biológicas Clemente Estable (IIBCE), Montevideo, Uruguay.; Neuroscience Division, IIBCE and Comparative Neuroanatomy-Associated Unit to the School of Sciences, UDELAR, Montevideo, Uruguay.; UMET, School of Sciences, UDELAR, Montevideo, Uruguay.; Neuroscience Division IIBCE, UDELAR, Montevideo, Uruguay.; Neurodegeneration Laboratory, Institut Pasteur Montevideo, Montevideo, Uruguay.

DOI: 10.1007/978-3-319-40764-7_11

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MeSH Terms: Alexander Disease, Amino Acid Metabolism, Inborn Errors, Antioxidants, Astrocytes, Brain, Brain Diseases, Metabolic, Ceruloplasmin, Diet, Disease Management, Glucose, Glutamate-Ammonia Ligase, Glutaryl-CoA Dehydrogenase, Hepatic Encephalopathy, Homeostasis, Humans, Iron Metabolism Disorders, Neurodegenerative Diseases, Neurogenesis, Niemann-Pick Disease, Type C, Pyruvate Carboxylase Deficiency Disease, Sorption Detoxification