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Mitochondrial processing peptidases.
Gakh O, Cavadini P, Isaya G. Gakh O, et al. Among authors: isaya g. Biochim Biophys Acta. 2002 Sep 2;1592(1):63-77. doi: 10.1016/s0167-4889(02)00265-3. Biochim Biophys Acta. 2002. PMID: 12191769 Review.
Reduction in frataxin causes progressive accumulation of mitochondrial damage.
Karthikeyan G, Santos JH, Graziewicz MA, Copeland WC, Isaya G, Van Houten B, Resnick MA. Karthikeyan G, et al. Among authors: isaya g. Hum Mol Genet. 2003 Dec 15;12(24):3331-42. doi: 10.1093/hmg/ddg349. Epub 2003 Oct 21. Hum Mol Genet. 2003. PMID: 14570713
Defining the Architecture of the Core Machinery for the Assembly of Fe-S Clusters in Human Mitochondria.
Gakh O, Ranatunga W, Galeano BK, Smith DS 4th, Thompson JR, Isaya G. Gakh O, et al. Among authors: isaya g. Methods Enzymol. 2017;595:107-160. doi: 10.1016/bs.mie.2017.07.003. Epub 2017 Aug 18. Methods Enzymol. 2017. PMID: 28882199
Architecture of the Human Mitochondrial Iron-Sulfur Cluster Assembly Machinery.
Gakh O, Ranatunga W, Smith DY 4th, Ahlgren EC, Al-Karadaghi S, Thompson JR, Isaya G. Gakh O, et al. Among authors: isaya g. J Biol Chem. 2016 Sep 30;291(40):21296-21321. doi: 10.1074/jbc.M116.738542. Epub 2016 Aug 12. J Biol Chem. 2016. PMID: 27519411 Free PMC article.
Architecture of the Yeast Mitochondrial Iron-Sulfur Cluster Assembly Machinery: THE SUB-COMPLEX FORMED BY THE IRON DONOR, Yfh1 PROTEIN, AND THE SCAFFOLD, Isu1 PROTEIN.
Ranatunga W, Gakh O, Galeano BK, Smith DY 4th, Söderberg CA, Al-Karadaghi S, Thompson JR, Isaya G. Ranatunga W, et al. Among authors: isaya g. J Biol Chem. 2016 May 6;291(19):10378-98. doi: 10.1074/jbc.M115.712414. Epub 2016 Mar 3. J Biol Chem. 2016. PMID: 26941001 Free PMC article.
Iron-sulfur cluster synthesis, iron homeostasis and oxidative stress in Friedreich ataxia.
Vaubel RA, Isaya G. Vaubel RA, et al. Among authors: isaya g. Mol Cell Neurosci. 2013 Jul;55:50-61. doi: 10.1016/j.mcn.2012.08.003. Epub 2012 Aug 11. Mol Cell Neurosci. 2013. PMID: 22917739 Free PMC article. Review.
Mutations in the dimer interface of dihydrolipoamide dehydrogenase promote site-specific oxidative damages in yeast and human cells.
Vaubel RA, Rustin P, Isaya G. Vaubel RA, et al. Among authors: isaya g. J Biol Chem. 2011 Nov 18;286(46):40232-45. doi: 10.1074/jbc.M111.274415. Epub 2011 Sep 19. J Biol Chem. 2011. PMID: 21930696 Free PMC article.
Leucine biosynthesis regulates cytoplasmic iron-sulfur enzyme biogenesis in an Atm1p-independent manner.
Bedekovics T, Li H, Gajdos GB, Isaya G. Bedekovics T, et al. Among authors: isaya g. J Biol Chem. 2011 Nov 25;286(47):40878-88. doi: 10.1074/jbc.M111.270082. Epub 2011 Sep 16. J Biol Chem. 2011. PMID: 21926174 Free PMC article.
Normal and Friedreich ataxia cells express different isoforms of frataxin with complementary roles in iron-sulfur cluster assembly.
Gakh O, Bedekovics T, Duncan SF, Smith DY 4th, Berkholz DS, Isaya G. Gakh O, et al. Among authors: isaya g. J Biol Chem. 2010 Dec 3;285(49):38486-501. doi: 10.1074/jbc.M110.145144. Epub 2010 Oct 2. J Biol Chem. 2010. PMID: 20889968 Free PMC article.
Oligomeric yeast frataxin drives assembly of core machinery for mitochondrial iron-sulfur cluster synthesis.
Li H, Gakh O, Smith DY 4th, Isaya G. Li H, et al. Among authors: isaya g. J Biol Chem. 2009 Aug 14;284(33):21971-80. doi: 10.1074/jbc.M109.011197. Epub 2009 Jun 2. J Biol Chem. 2009. PMID: 19491103 Free PMC article.
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