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Inhibition of colony-stimulating factor 1 receptor early in disease ameliorates motor deficits in SCA1 mice.

Journal of neuroinflammation | 2017

Polyglutamine (polyQ) expansion in the protein Ataxin-1 (ATXN1) causes spinocerebellar ataxia type 1 (SCA1), a fatal dominantly inherited neurodegenerative disease characterized by motor deficits, cerebellar neurodegeneration, and gliosis. Currently, there are no treatments available to delay or ameliorate SCA1. We have examined the effect of depleting microglia during the early stage of disease by using PLX, an inhibitor of colony-stimulating factor 1 receptor (CSFR1), on disease severity in a mouse model of SCA1.

Pubmed ID: 28545543 RIS Download

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Associated grants

  • Agency: NIGMS NIH HHS, United States
    Id: T32 GM008471

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Fiji (tool)

RRID:SCR_002285

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RRID:SCR_014246

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