Manganese is the link between frataxin and iron-sulfur deficiency in the yeast model of Friedreich ataxia

The Journal of Biological Chemistry
Verónica IrazustaJordi Tamarit

Abstract

Friedreich ataxia is a human neurodegenerative and myocardial disease caused by decreased expression of the mitochondrial protein frataxin. Proteomic analysis of the mutant yeast model of Friedreich ataxia presented in this paper reveals that these cells display increased amounts of proteins involved in antioxidant defenses, including manganese-superoxide dismutase. This enzyme shows, however, lower activity than that found in wild type cells. Our results indicate that this lack of activity is a consequence of cellular manganese deficiency, because in manganese-supplemented cultures, cell manganese content, and manganese-superoxide dismutase activity were restored. One of the hallmarks of Friedreich ataxia is the decreased activity of iron/sulfur-containing enzymes. The activities of four enzymes of this group (aconitase, glutamate synthase, succinate dehydrogenase, and isopropylmalate dehydratase) have been analyzed for the effects of manganese supplementation. Enzyme activities were recovered by manganese treatment, except for aconitase, for which, a specific interaction with frataxin has been demonstrated previously. Similar results were obtained when cells were grown in iron-limited media suggesting that manganese-superoxid...Continue Reading

References

Jan 1, 1988·Methods in Enzymology·G B Kohlhaw
Jan 1, 1995·Annual Review of Biochemistry·I Fridovich
Mar 1, 1995·Molecular and Cellular Biology·P J LapinskasV C Culotta
Feb 22, 2000·The Journal of Biological Chemistry·S G ParkI H Kim
Jun 15, 2000·The Journal of Biological Chemistry·E CabiscolJ Ros
Oct 13, 2000·Human Molecular Genetics·P CavadiniG Isaya
Sep 14, 2001·Human Molecular Genetics·M M SantosM Pandolfo
Oct 9, 2001·Human Molecular Genetics·K Chantrel-GroussardP Rustin
Apr 16, 2002·Molecular Biology of the Cell·María Teresa Rodríguez-ManzanequeEnrique Herrero
Jun 18, 2002·Trends in Molecular Medicine·Agnès RötigPierre Rustin
Jul 27, 2002·Annual Review of Microbiology·Anthony Van HoJerry Kaplan
Oct 2, 2002·Human Molecular Genetics·Geoffrey DubyThomas Lutz
Dec 31, 2002·Brain Research. Brain Research Reviews·Atsushi Takeda
Mar 1, 2003·Science·Hugo AguilaniuThomas Nyström
May 5, 2004·The Journal of Biological Chemistry·Taejin Yoon, J A Cowan
Jul 30, 2004·Human Molecular Genetics·Alessandro CampanellaSonia Levi
Dec 24, 2004·Human Molecular Genetics·Hervé SeznecHélène Puccio
Jan 12, 2005·Biochemistry·Heather A O'NeillGrazia Isaya
Feb 5, 2005·Science·Xin Jie ChenRonald A Butow
Mar 16, 2005·The Journal of Biological Chemistry·Yan ZhangAndrew Dancis
Apr 21, 2005·Proceedings of the National Academy of Sciences of the United States of America·Anne-Laure BulteauLuke I Szweda
Jun 18, 2005·Human Molecular Genetics·Pilar González-CaboFrancesc Palau
Jan 13, 2006·Analytical Biochemistry·Jordi TamaritJoaquim Ros

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Citations

Oct 20, 2010·The Journal of Biological Chemistry·Armando Moreno-CermeñoJordi Tamarit
Sep 18, 2010·The Journal of Biological Chemistry·Maria José Rodriguez-ColmanElisa Cabiscol
Feb 17, 2010·Antioxidants & Redox Signaling·Renata SantosEmmanuel Lesuisse
Jan 12, 2007·Biochemistry and Cell Biology = Biochimie Et Biologie Cellulaire·P CourvilleM F M Cellier
Apr 12, 2011·Biometals : an International Journal on the Role of Metal Ions in Biology, Biochemistry, and Medicine·Mark R Bleackley, Ross T A Macgillivray
Mar 7, 2012·Biometals : an International Journal on the Role of Metal Ions in Biology, Biochemistry, and Medicine·Verónica IrazustaLucía I C de Figueroa
May 10, 2011·Brain Research Reviews·Daniele Marmolino
Jan 8, 2008·Biochimica Et Biophysica Acta·Enrique HerreroElisa Cabiscol
Jun 29, 2007·Molecular Genetics and Metabolism·Ngolela E BabadyHélène Puccio
Aug 13, 2013·Oxidative Medicine and Cellular Longevity·Cláudio M Gomes, Renata Santos
Mar 23, 2010·The Biochemical Journal·Jianxin LuHuangen Ding
Oct 9, 2013·Biochimica Et Biophysica Acta·Armando Moreno-CermeñoJoaquim Ros
Apr 4, 2013·Free Radical Biology & Medicine·Sandra CasaniNuria Paricio
Dec 28, 2007·Biochimica Et Biophysica Acta·Isabella ZanellaPaolo Arosio
Jul 15, 2015·PloS One·Pablo Calap-QuintanaMaría José Martínez-Sebastián
Jun 15, 2016·Free Radical Biology & Medicine·Jordi TamaritJoaquim Ros
Jun 3, 2017·Metallomics : Integrated Biometal Science·T H L HanN T Ha-Duong
Dec 3, 2017·FEBS Letters·Federica LupoliAnnalisa Pastore
Aug 17, 2006·Critical Reviews in Biochemistry and Molecular Biology·Krisztina Z BenczeTimothy L Stemmler
Dec 15, 2006·FASEB Journal : Official Publication of the Federation of American Societies for Experimental Biology·José V LlorensMaria D Moltó
Dec 5, 2019·Metallomics : Integrated Biometal Science·Johana Vásquez-ProcopioFanis Missirlis
Jun 14, 2006·The Journal of Biological Chemistry·Yan ZhangAndrew Dancis
Dec 21, 2016·Journal of Inherited Metabolic Disease·Lisa G RileyJohn Christodoulou
Mar 7, 2021·IUBMB Life·Jordi TamaritJoaquim Ros
Jul 12, 2021·Orphanet Journal of Rare Diseases·Eleonora BonaventuraRoberta Battini
Jul 1, 2008·Free Radical Biology & Medicine·Ma Alba SorollaElisa Cabiscol

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