Neuroprotective Effects of σ₂R/TMEM97 Receptor Modulators in the Neuronal Model of Huntington's Disease.

ABSTRACT: Huntington's disease (HD) is a genetic neurodegenerative disease caused by an expanded CAG repeat in the Huntingtin (HTT) gene that encodes for an expanded polyglutamine (polyQ) repeat in exon-1 of the human mutant huntingtin (mHTT) protein. The presence of this polyQ repeat results in neuronal degeneration, for which there is no cure or treatment that modifies disease progression. In previous studies, we have shown that small molecules that bind selectively to σ₂R/TMEM97 can have significant neuroprotective effects in models of Alzheimer's disease, traumatic brain injury, and several other neurodegenerative diseases. In the present work, we extend these investigations and show that certain σ₂R/TMEM97-selective ligands decrease mHTT-induced neuronal toxi

SUBMITTER: Jin J 

PROVIDER: S-EPMC9547941 | biostudies-literature | 2022 Oct

REPOSITORIES: biostudies-literature