Glial mitochondrial function and dysfunction in health and neurodegeneration.

Glial mitochondrial function and dysfunction in health and neurodegeneration.

Publication date: Oct 31, 2019

Mitochondria play essential metabolic roles in neural cells. Mitochondrial dysfunction has profound effects on the brain. In primary mitochondrial diseases, mutations that impair specific oxidative phosphorylation (OXPHOS) proteins or OXPHOS assembly factors lead to isolated biochemical defects and a heterogeneous group of clinical phenotypes, including mitochondrial encephalopathies. A broader defect of OXPHOS function, due to mutations in proteins involved in mitochondrial DNA maintenance, mitochondrial biogenesis, or mitochondrial tRNAs can also underlie severe mitochondrial encephalopathies. While primary mitochondrial dysfunction causes rare genetic forms of neurological disorders, secondary mitochondrial dysfunction is involved in the pathophysiology of some of the most common neurodegenerative disorders, including Alzheimer’s disease, Parkinson’s disease, Huntington’s disease, and amyotrophic lateral sclerosis. Many studies have investigated mitochondrial function and dysfunction in bulk central nervous system (CNS) tissue. However, the interpretation of these studies has been often complicated by the extreme cellular heterogeneity of the CNS, which includes many different types of neurons and glial cells. Because neurons are especially dependent on OXPHOS for ATP generation, mitochondrial dysfunction is thought to be directly involved in cell autonomous neuronal demise. Despite being metabolically more flexible than neurons, glial mitochondria also play an essential role in the function of the CNS, and have adapted specific metabolic and mitochondrial features to support their diversity of functions. This review analyzes our current understanding and the gaps in knowledge of mitochondrial properties of glia and how they affect neuronal functions, in health and disease.

McAvoy, K. and Kawamata, H. Glial mitochondrial function and dysfunction in health and neurodegeneration. 06745. 2019 Mol Cell Neurosci.

Concepts Keywords
Alzheimer Common neurodegenerative disorders
Amyotrophic Lateral Sclerosis Mitochondrial diseases
Assembly Glial mitochondrial dysfunction
ATP Generation Amyotrophic lateral sclerosis
Biochemical Secondary mitochondrial dysfunction
Brain Branches of biology
Central Nervous System Glial cells
Encephalopathies Mitochondria
Genetic Cell biology
Glia Molecular biology
Glial Mitochondrion
Glial Cells Mitochondrial disease
Heterogeneity Neurodegeneration
Huntington Neuron
Mitochondria Astrocyte
Mitochondrial Mitochondrial biogenesis
Mitochondrial Diseases
Neurodegenerative Disorders
Neurological Disorders
Oxidative Phosphorylation


Type Source Name
disease MESH mitochondrial diseases
pathway BSID Oxidative phosphorylation
disease MESH defects
disease MESH encephalopathies
pathway BSID Mitochondrial biogenesis
disease MESH neurological disorders
disease MESH neurodegenerative disorders
disease MESH amyotrophic lateral sclerosis
disease DOID amyotrophic lateral sclerosis


Original Article

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