Astrocyte Dysfunction in Developmental Neurometabolic Diseases

Adv Exp Med Biol. 2016:949:227-243. doi: 10.1007/978-3-319-40764-7_11.

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: Astrocyte dysfunction; Astrocytes; Myelin damage; Neurodegeneration; Neurodevelopmental diseases.

Publication types

  • Review

MeSH terms

  • Alexander Disease / diagnosis
  • Alexander Disease / metabolism
  • Alexander Disease / pathology
  • Alexander Disease / therapy
  • Amino Acid Metabolism, Inborn Errors / diagnosis*
  • Amino Acid Metabolism, Inborn Errors / metabolism
  • Amino Acid Metabolism, Inborn Errors / pathology
  • Amino Acid Metabolism, Inborn Errors / therapy*
  • Antioxidants / therapeutic use
  • Astrocytes / drug effects
  • Astrocytes / metabolism
  • Astrocytes / pathology*
  • Brain / drug effects
  • Brain / metabolism
  • Brain / pathology*
  • Brain Diseases, Metabolic / diagnosis*
  • Brain Diseases, Metabolic / metabolism
  • Brain Diseases, Metabolic / pathology
  • Brain Diseases, Metabolic / therapy*
  • Ceruloplasmin / deficiency
  • Ceruloplasmin / metabolism
  • Diet / methods
  • Disease Management
  • Glucose / therapeutic use
  • Glutamate-Ammonia Ligase / deficiency
  • Glutamate-Ammonia Ligase / metabolism
  • Glutaryl-CoA Dehydrogenase / deficiency*
  • Glutaryl-CoA Dehydrogenase / metabolism
  • Hepatic Encephalopathy / diagnosis
  • Hepatic Encephalopathy / metabolism
  • Hepatic Encephalopathy / pathology
  • Hepatic Encephalopathy / therapy
  • Homeostasis
  • Humans
  • Iron Metabolism Disorders / diagnosis
  • Iron Metabolism Disorders / metabolism
  • Iron Metabolism Disorders / pathology
  • Iron Metabolism Disorders / therapy
  • Neurodegenerative Diseases / diagnosis
  • Neurodegenerative Diseases / metabolism
  • Neurodegenerative Diseases / pathology
  • Neurodegenerative Diseases / therapy
  • Neurogenesis / drug effects
  • Niemann-Pick Disease, Type C / diagnosis
  • Niemann-Pick Disease, Type C / metabolism
  • Niemann-Pick Disease, Type C / pathology
  • Niemann-Pick Disease, Type C / therapy
  • Pyruvate Carboxylase Deficiency Disease / diagnosis
  • Pyruvate Carboxylase Deficiency Disease / metabolism
  • Pyruvate Carboxylase Deficiency Disease / pathology
  • Pyruvate Carboxylase Deficiency Disease / therapy
  • Sorption Detoxification

Substances

  • Antioxidants
  • Ceruloplasmin
  • Glutaryl-CoA Dehydrogenase
  • Glutamate-Ammonia Ligase
  • Glucose

Supplementary concepts

  • Familial apoceruloplasmin deficiency
  • Glutamine deficiency, congenital
  • Glutaric Acidemia I