Contribution of Glial Cells to Polyglutamine Diseases: Observations from Patients and Mouse Models.

ABSTRACT: Neurodegenerative diseases are broadly characterized neuropathologically by the degeneration of vulnerable neuronal cell types in a specific brain region. The degeneration of specific cell types has informed on the various phenotypes/clinical presentations in someone suffering from these diseases. Prominent neurodegeneration of specific neurons is seen in polyglutamine expansion diseases including Huntington's disease (HD) and spinocerebellar ataxias (SCA). The clinical manifestations observed in these diseases could be as varied as the abnormalities in motor function observed in those who have Huntington's disease (HD) as demonstrated by a chorea with substantial degeneration of striatal medium spiny neurons (MSNs) or those with various forms of spinocerebellar ataxia (SCA) with an ataxic

SUBMITTER: Cvetanovic M 

PROVIDER: S-EPMC10119372 | biostudies-literature | 2023 Jan

REPOSITORIES: biostudies-literature