Brain mitochondrial iron accumulates in Huntington's disease, mediates mitochondrial dysfunction, and can be removed pharmacologically

Sonal Agrawal, Julia Fox, Baskaran Thyagarajan, Jonathan Fox, Free Radical Biology and Medicine, Available online 4 April 2018, ISSN 0891-5849, doi:10.1016/j.freeradbiomed.2018.04.002.

We demonstrate here that human Huntington's disease and mouse model HD (12-week R6/2 and 12-month YAC128) brains accumulated mitochondrial iron and showed increased expression of iron uptake protein mitoferrin 2 and decreased iron-sulfur cluster synthesis protein frataxin.