Astrocyte Dysfunction in Developmental Neurometabolic Diseases
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 astrocyt
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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.
Keywords Astrocytes Astrocyte dysfunction Neurodegeneration Myelin damage
Neurodevelopmental diseases
S. Olivera-Bravo (&) E. Isasi Cellular and Molecular Neurobiology, Instituto de Investigaciones Biológicas Clemente Estable (IIBCE), Montevideo, Uruguay e-mail: [email protected] E. Isasi e-mail: [email protected] A. Fernández Neuroscience Division, IIBCE and Comparative Neuroanatomy-Associated Unit to the School of Sciences, UDELAR, Montevideo, Uruguay e-mail: [email protected] G. Casanova UMET, School of Sciences, UDELAR, Montevideo, Uruguay e-mail: [email protected] J.C. Rosillo Neuroscience Division IIBCE, UDELAR, Montevideo, Uruguay e-mail: [email protected] L. Barbeito Neurodegeneration Laboratory, Institut Pasteur Montevideo, Montevideo, Uruguay e-mail: [email protected] © Springer International Publishing Switzerland 2016 R. von Bernhardi (ed.), Glial Cells in Health and Disease of the CNS, Advances in Experimental Medicine and Biology 949, DOI 10.1007/978-3-319-40764-7_11
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Abbreviations ALS AQP4 BBB GA GA-I GCDH GDNF GFAP Icv IEM MAPK MLC NPC NVU PC VWM
Amyotrophic Lateral Sclerosis Aquaporin 4 Blood–Brain Barrier Glutaric Acid Glutaric Acidemia Type I Glutaryl CoA Dehydrogenase Glial-Derived Neurotrophic Factor Glial Fibrillary Acidic Protein Intracerebroventricular Inborn Errors of Metabolism Mitogen-Activated Protein Kinases Megalencephalic Leukoencephalopathy with Subcortical Cysts Niemann-Pick type C Disease Neurovascular Unit Pyruvate Carboxylase Vanishing White Matter
Introduction to Astrocyte Functions As previously discussed in Chapters “Glial Cells and Integrity of the Nervous System,” “Pharmacological Tools to Study the Role of Astrocytes in Neural Network Functions,” and “Role of Astrocytes in Central Respiratory Chemoreception,” astrocytes are indispensable for neuronal survival and activity. They play many key regulatory functions in brain homeostasis, including glutamate uptake that is vital for maintaining a physiological balance and protecting against neurotoxicity (Rothstein et al. 1996). Astrocytes are the only cells in the CNS that store and process glycogen. They thus contribute to influx of glucose and energy intermediates into neurons, and are also a significant source of neurotrophins and antioxidant defenses (Maragakis and Rothstein 20
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