Estrogen-related receptor gamma is a regulator of mitochondrial, autophagy, and immediate-early gene programs in spiny projection neurons: Relevance for transcriptional changes in Huntington disease.

ABSTRACT: Mitochondrial dysfunction, transcriptional dysregulation, and protein aggregation are hallmarks of multiple neurodegenerative disorders, including Huntington's disease (HD). Strategies are needed to counteract these processes to restore neuronal health and function in HD. Recent evidence indicates that the transcription factor estrogen-related receptor gamma (ERRγ/Esrrg) is required for normal expression of mitochondrial, synaptic, and autophagy genes in neurons. Further, overexpression of Esrrg in dopaminergic neurons reduces synuclein load in the pre-formed fibril model of synucleinopathy. For these reasons, we sought to understand ERRγ's role in transcriptional regulation in spiny projection neurons (SPNs), one of the neuronal populations vulnerable to transcriptional dysregulation, mit

SUBMITTER: Fox SN 

PROVIDER: S-EPMC12208617 | biostudies-literature | 2025 Mar

REPOSITORIES: biostudies-literature