Open AccessRemoval of the Mitochondrial Fission Factor Mff Exacerbates Neuronal Loss and Neurological Phenotypes in a Huntington's Disease Mouse Model
Author(s) -
Yong Moon,
Hsiuchen Chen,
David C. Chan
Publication year - 2018
Publication title -
plos currents
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.282
H-Index - 49
ISSN - 2157-3999
DOI - 10.1371/currents.hd.a4e15b80c4915c828d39754942c6631f
Subject(s) - mitochondrial fission , phenotype , huntington's disease , mitochondrion , biology , genetically modified mouse , mutant , microbiology and biotechnology , neuroinflammation , disease , neuroscience , transgene , pathology , bioinformatics , medicine , genetics , gene
Excessive mitochondrial fission has been associated with several neurodegenerative diseases, including Huntington's disease (HD). Consequently, mitochondrial dynamics has been suggested to be a promising therapeutic target for Huntington's disease. Mitochondrial fission depends on recruitment of Drp1 to mitochondria, and Mff (mitochondrial fission factor) is one of the key adaptor proteins for this process. Removal of Mff therefore greatly reduces mitochondrial fission. Here we investigate whether removal of Mff can mitigate HD-associated pathologies in HD transgenic mice (R6/2) expressing mutant Htt.