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Glial Cells in Health and Disease of the CNS pp 227–243Cite as

Astrocyte Dysfunction in Developmental Neurometabolic Diseases

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Part of the book series: Advances in Experimental Medicine and Biology ((AEMB,volume 949))

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.

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Abbreviations

ALS:

Amyotrophic Lateral Sclerosis

AQP4:

Aquaporin 4

BBB:

Blood–Brain Barrier

GA:

Glutaric Acid

GA-I:

Glutaric Acidemia Type I

GCDH:

Glutaryl CoA Dehydrogenase

GDNF:

Glial-Derived Neurotrophic Factor

GFAP:

Glial Fibrillary Acidic Protein

Icv:

Intracerebroventricular

IEM:

Inborn Errors of Metabolism

MAPK:

Mitogen-Activated Protein Kinases

MLC:

Megalencephalic Leukoencephalopathy with Subcortical Cysts

NPC:

Niemann-Pick type C Disease

NVU:

Neurovascular Unit

PC:

Pyruvate Carboxylase

VWM:

Vanishing White Matter

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Author information

Authors and Affiliations

  1. Cellular and Molecular Neurobiology, Instituto de Investigaciones Biológicas Clemente Estable (IIBCE), Montevideo, Uruguay

    Silvia Olivera-Bravo & Eugenia Isasi

  2. Neuroscience Division, IIBCE and Comparative Neuroanatomy-Associated Unit to the School of Sciences, UDELAR, Montevideo, Uruguay

    Anabel Fernández

  3. UMET, School of Sciences, UDELAR, Montevideo, Uruguay

    Gabriela Casanova

  4. Neuroscience Division IIBCE, UDELAR, Montevideo, Uruguay

    Juan Carlos Rosillo

  5. Neurodegeneration Laboratory, Institut Pasteur Montevideo, Montevideo, Uruguay

    Luigi Barbeito

Authors
  1. Silvia Olivera-Bravo
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  2. Eugenia Isasi
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  3. Anabel Fernández
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  4. Gabriela Casanova
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  5. Juan Carlos Rosillo
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  6. Luigi Barbeito
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Corresponding author

Correspondence to Silvia Olivera-Bravo .

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Editors and Affiliations

  1. Faculty of Medicine Professor of Neurology, Pontificia Universidad Católica de Chile, Santiago, Chile

    Rommy von Bernhardi

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Olivera-Bravo, S., Isasi, E., Fernández, A., Casanova, G., Rosillo, J.C., Barbeito, L. (2016). Astrocyte Dysfunction in Developmental Neurometabolic Diseases. In: von Bernhardi, R. (eds) Glial Cells in Health and Disease of the CNS. Advances in Experimental Medicine and Biology, vol 949. Springer, Cham. https://doi.org/10.1007/978-3-319-40764-7_11

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