Article
Reduction in frataxin causes progressive accumulation of mitochondrial damage.
Human molecular genetics - 15 Dec 2003
Karthikeyan Gopalakrishnan, Santos Janine H, Graziewicz Maria A, Copeland William C, Isaya Grazia, Van Houten Bennett, Resnick Michael A
Abstract excerpt
Frataxin protein controls iron availability in mitochondria and reduced levels lead to the human disease, Friedreich's ataxia (FRDA). The molecular aspects of disease progression are not well understood. We developed a highly regulatable promoter system for expressing frataxin in yeast to address...
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