Huntingtin preserves mitochondrial genome integrity in neurons, which is impaired in Huntingtons disease
Subrata Pradhan, Sagar Gaikwad, Chi-Lin Tsai, Charlene Smith-Geater, Nan Zhang, Keegan M. Bush, Anirban Chakraborty, Subo Yuan, Sanjeev Choudhary, Christopher Dirk Keene, Lisa Ellerby, Tapas K. Hazra, Albert R. La Spada, Yogesh P. Wairkar, Tatsuo Ashizawa, , Partha Sarathi Sarkar
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.
bioRxiv · Jul 24, 2025 · PreprintNeuroscience