Friday, April 6, 2018

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.