Role of Microglia in Ataxias.

Abstract:

:Microglia, the resident macrophages of the central nervous system, critically influence neural function during development and in adulthood. Microglia are also profoundly sensitive to insults to the brain to which they respond with process of activation that includes spectrum of changes in morphology, function, and gene expression. Ataxias are a class of neurodegenerative diseases characterized by motor discoordination and predominant cerebellar involvement. In case of inherited forms of ataxia, mutant proteins are expressed throughout the brain and it is unclear why cerebellum is particularly vulnerable. Recent studies demonstrated that cerebellar microglia have a uniquely hyper-vigilant immune phenotype compared to microglia from other brain regions. These findings may indicate that microglia actively contribute to cerebellar vulnerability in ataxias. Here we review current knowledge about cerebellar microglia, their activation, and their role in the pathogenesis of ataxias. In addition, we briefly review advantages and disadvantages of several experimental approaches available to study microglia.

journal_name

J Mol Biol

authors

Ferro A,Sheeler C,Rosa JG,Cvetanovic M

doi

10.1016/j.jmb.2019.01.016

subject

Has Abstract

pub_date

2019-04-19 00:00:00

pages

1792-1804

issue

9

eissn

0022-2836

issn

1089-8638

pii

S0022-2836(19)30025-7

journal_volume

431

pub_type

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