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Mitochondrial processing peptidases.
Gakh O, Cavadini P, Isaya G. Gakh O, et al. 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.
PMPCA mutations cause abnormal mitochondrial protein processing in patients with non-progressive cerebellar ataxia.
Jobling RK, Assoum M, Gakh O, Blaser S, Raiman JA, Mignot C, Roze E, Dürr A, Brice A, Lévy N, Prasad C, Paton T, Paterson AD, Roslin NM, Marshall CR, Desvignes JP, Roëckel-Trevisiol N, Scherer SW, Rouleau GA, Mégarbané A, Isaya G, Delague V, Yoon G. Jobling RK, et al. Among authors: gakh o. Brain. 2015 Jun;138(Pt 6):1505-17. doi: 10.1093/brain/awv057. Epub 2015 Mar 25. Brain. 2015. PMID: 25808372 Free PMC article.
Assembly of the iron-binding protein frataxin in Saccharomyces cerevisiae responds to dynamic changes in mitochondrial iron influx and stress level.
Gakh O, Smith DY 4th, Isaya G. Gakh O, et al. J Biol Chem. 2008 Nov 14;283(46):31500-10. doi: 10.1074/jbc.M805415200. Epub 2008 Sep 9. J Biol Chem. 2008. PMID: 18784075 Free PMC article.
Supramolecular assemblies of human frataxin are formed via subunit-subunit interactions mediated by a non-conserved amino-terminal region.
O'Neill HA, Gakh O, Isaya G. O'Neill HA, et al. Among authors: gakh o. J Mol Biol. 2005 Jan 21;345(3):433-9. doi: 10.1016/j.jmb.2004.10.074. J Mol Biol. 2005. PMID: 15581888
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. 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. 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: gakh o. 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.
Missense mutations linked to friedreich ataxia have different but synergistic effects on mitochondrial frataxin isoforms.
Li H, Gakh O, Smith DY 4th, Ranatunga WK, Isaya G. Li H, et al. Among authors: gakh o. J Biol Chem. 2013 Feb 8;288(6):4116-27. doi: 10.1074/jbc.M112.435263. Epub 2012 Dec 26. J Biol Chem. 2013. PMID: 23269675 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. 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: gakh o. 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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