Article
H63D HFE genotype accelerates disease progression in animal models of amyotrophic lateral sclerosis.
Biochimica et biophysica acta - 1 Dec 2014
Nandar Wint, Neely Elizabeth B, Simmons Zachary, Connor James R
Abstract excerpt
H63D HFE is associated with iron dyshomeostasis and oxidative stress; each of which plays an important role in amyotrophic lateral sclerosis (ALS) pathogenesis. To examine the role of H63D HFE in ALS, we generated a double transgenic mouse line (SOD1/H67D) carrying the H67D HFE (homologue of human H63D) and SOD1(G93A) mutations. We found double transgenic mice have shorter survival and accelerated disease...
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