Astrocyte Dysfunction in Developmental Neurometabolic Diseases

  • Silvia Olivera-BravoEmail author
  • Eugenia Isasi
  • Anabel Fernández
  • Gabriela Casanova
  • Juan Carlos Rosillo
  • Luigi Barbeito
Part of the Advances in Experimental Medicine and Biology book series (AEMB, volume 949)


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.


Astrocytes Astrocyte dysfunction Neurodevelopmental diseases Neurodegeneration Myelin damage 



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




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


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Copyright information

© Springer International Publishing Switzerland 2016

Authors and Affiliations

  • Silvia Olivera-Bravo
    • 1
    Email author
  • Eugenia Isasi
    • 1
  • Anabel Fernández
    • 2
  • Gabriela Casanova
    • 3
  • Juan Carlos Rosillo
    • 4
  • Luigi Barbeito
    • 5
  1. 1.Cellular and Molecular NeurobiologyInstituto de Investigaciones Biológicas Clemente Estable (IIBCE)MontevideoUruguay
  2. 2.Neuroscience Division, IIBCE and Comparative Neuroanatomy-Associated Unit to the School of SciencesUDELARMontevideoUruguay
  3. 3.UMET, School of SciencesUDELARMontevideoUruguay
  4. 4.Neuroscience Division IIBCEUDELARMontevideoUruguay
  5. 5.Neurodegeneration LaboratoryInstitut Pasteur MontevideoMontevideoUruguay

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