Myelinating Glia: Potential Therapeutic Targets in Polyglutamine Spinocerebellar Ataxias

Human studies, in combination with animal and cellular models, support glial cells as both major contributors to neurodegenerative diseases and promising therapeutic targets. Among glial cells, oligodendrocytes and Schwann cells are the myelinating glial cells of the central and peripheral nervous s...

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Bibliographic Details
Published in:Cells (Basel, Switzerland) Switzerland), 2023-02, Vol.12 (4), p.601
Main Authors: Putka, Alexandra F, Mato, Juan P, McLoughlin, Hayley S
Format: Article
Language:English
Subjects:
Animal models
Animals
Anisotropy
Ataxia
Brain research
Cell culture
Disease
Drug targeting
Drug therapy
Glial cells
Health aspects
Humans
Lipids
Metabolism
Metabolites
Methods
Nervous system
neurodegeneration
Neurodegenerative diseases
Neuroglia
Neuronal-glial interactions
Neurons
oligodendrocyte
Oligodendrocytes
Patients
Peptides
Peripheral nerves
Peripheral neuropathy
Polyglutamine
Polyglutamine diseases
Proteins
Review
Schwann cell
Schwann Cells
Spinocerebellar ataxia
Spinocerebellar Ataxias
Therapeutic applications
Therapeutic targets
Trinucleotide repeat diseases
white matter
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Summary:Human studies, in combination with animal and cellular models, support glial cells as both major contributors to neurodegenerative diseases and promising therapeutic targets. Among glial cells, oligodendrocytes and Schwann cells are the myelinating glial cells of the central and peripheral nervous system, respectively. In this review, we discuss the contributions of these central and peripheral myelinating glia to the pathomechanisms of polyglutamine (polyQ) spinocerebellar ataxia (SCA) types 1, 2, 3, 6, 7, and 17. First, we highlight the function of oligodendrocytes in healthy conditions and how they are disrupted in polyQ SCA patients and diseased model systems. We then cover the role of Schwann cells in peripheral nerve function and repair as well as their possible role in peripheral neuropathy in polyQ SCAs. Finally, we discuss potential polyQ SCA therapeutic interventions in myelinating glial.
ISSN:2073-4409
2073-4409
DOI:10.3390/cells12040601