Charlene Smith-Geater

The paper shows that Huntingtin (HTT) helps maintain mitochondrial DNA integrity by organizing a repair complex, which is disrupted by mutant HTT in Huntington's disease, leading to DNA damage and motor deficits in a fly model.

This study compares the effects of HTT knockout and CAG repeat expansion in cortical neurons, identifying shared and distinct biological pathways involved in HD.

This study identifies a mechanism where HTT recruits BRG1 and NHEJ components to repair transcription-associated DSBs in neurons, and shows that mutant HTT impairs this process, contributing to HD pathology.

This study shows that BDNF and TRiC-inspired reagents can rescue synaptic deficits in HD mouse models.

This study uses cryoET and proteomics to identify mitochondrial and organelle abnormalities in HD models, showing potential for early diagnosis and therapy development.