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Full-Text Articles in Other Biochemistry, Biophysics, and Structural Biology
Yeast And Human Frataxin Are Processed To Mature Form In Two Sequential Steps By The Mitochondrial Processing Peptidase, Steven S. Branda, Patrizia Cavadini, Jiri Adamec, Frantisek Kalousek, Franco Taroni, Grazia Isaya
Yeast And Human Frataxin Are Processed To Mature Form In Two Sequential Steps By The Mitochondrial Processing Peptidase, Steven S. Branda, Patrizia Cavadini, Jiri Adamec, Frantisek Kalousek, Franco Taroni, Grazia Isaya
Department of Biochemistry: Faculty Publications
Frataxin is a nuclear-encoded mitochondrial protein which is deficient in Friedreich’s ataxia, a hereditary neurodegenerative disease. Yeast mutants lacking the yeast frataxin homologue (Yfh1p) show iron accumulation in mitochondria and increased sensitivity to oxidative stress, suggesting that frataxin plays a critical role in mitochondrial iron homeostasis and free radical toxicity. Both Yfh1p and frataxin are synthesized as larger precursor molecules that, upon import into mitochondria, are subject to two proteolytic cleavages, yielding an intermediate and a mature size form. A recent study found that recombinant rat mitochondrial processing peptidase (MPP) cleaves the mouse frataxin precursor to the intermediate but not …
Archaeal Aminoacyl-Trna Synthesis: Unique Determinants Of A Universal Genetic Code?, Michael Ibba, A. W. Curnow, J. Bono, P. A. Rosa, C. R. Woese, D. Söll
Archaeal Aminoacyl-Trna Synthesis: Unique Determinants Of A Universal Genetic Code?, Michael Ibba, A. W. Curnow, J. Bono, P. A. Rosa, C. R. Woese, D. Söll
Biology, Chemistry, and Environmental Sciences Faculty Articles and Research
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