Mt-Hsp70 homolog, Ssc2p, required for maturation of yeast frataxin and mitochondrial iron homeostasis.

The Journal of Biological Chemistry
S A KnightA Dancis

Abstract

Here we show that the yeast mitochondrial chaperone Ssc2p, a homolog of mt-Hsp70, plays a critical role in mitochondrial iron homeostasis. Yeast with ssc2-1 mutations were identified by a screen for altered iron-dependent gene regulation and mitochondrial dysfunction. These mutants exhibit increased cellular iron uptake, and the iron accumulates exclusively within mitochondria. Yfh1p is homologous to frataxin, the human protein implicated in the neurodegenerative disease, Friedreich's ataxia. Like mutants of yfh1, ssc2-1 mutants accumulate vast quantities of iron in mitochondria. Furthermore, using import studies with isolated mitochondria, we demonstrate a specific role for Ssc2p in the maturation of Yfh1p within this organelle. This function for a mitochondrial Hsp70 chaperone is likely to be conserved, implying that a human homolog of Ssc2p may be involved in iron homeostasis and in neurodegenerative disease.

References

May 1, 1990·Molecular and Cellular Biology·A DancisJ G Barriocanal
Oct 1, 1993·The Journal of Cell Biology·B D GambillN Pfanner
Dec 15, 1996·Experientia·T Langer, W Neupert
Jan 27, 1998·The Journal of Biological Chemistry·N B SepuriD Pain

❮ Previous
Next ❯

Citations

Jul 13, 1999·Annals of Neurology·D WaldvogelM Hallett
Jul 24, 2003·Journal of Molecular Biology·Maja ChloupkováDavid M Koeller
Aug 23, 2002·Biochimica Et Biophysica Acta·Wolfgang Voos, Karin Röttgers
Jun 22, 2002·Current Opinion in Genetics & Development·Hélène Puccio, Michel Koenig
Aug 1, 2000·Trends in Biochemical Sciences·R Lill, G Kispal
Jun 11, 2002·Methods : a Companion to Methods in Enzymology·Patrizia CavadiniGrazia Isaya
Apr 26, 2003·Current Opinion in Chemical Biology·Jeverson Frazzon, Dennis R Dean
Apr 2, 2005·Nature Reviews. Molecular Cell Biology·Tracey A Rouault, Wing-Hang Tong
Jul 21, 2000·European Journal of Biochemistry·S TaketaniY Nakahashi
Sep 1, 1999·Proceedings of the National Academy of Sciences of the United States of America·B SchilkeE Craig
Nov 30, 2012·The Journal of Biological Chemistry·Vinay A PatilMiriam L Greenberg
May 27, 1999·The Proceedings of the Nutrition Society·J J PowellR P Thompson
Mar 11, 2008·Antioxidants & Redox Signaling·Elizabeth L MacKenzieYoshiaki Tsuji
Feb 17, 2010·Antioxidants & Redox Signaling·Renata SantosEmmanuel Lesuisse
Oct 10, 2008·Molecular Biology of the Cell·Amy J AndrewElizabeth A Craig
Feb 11, 2012·Molecular Biology of the Cell·Alex D SheftelRoland Lill
Jan 25, 2013·Molecular Biology of the Cell·Adinarayana MaradaNaresh Babu V Sepuri
Apr 23, 2011·Nucleic Acids Research·María Del Carmen Díaz de la LozaRalf Erik Wellinger
Apr 25, 2000·Molecular and Cellular Biology·C VoisineE A Craig
May 11, 2000·Molecular and Cellular Biology·L T Jensen, V C Culotta
Jun 13, 2012·Microbiology and Molecular Biology Reviews : MMBR·Jacob VergheseKevin A Morano
Jun 6, 2000·Microbiology and Molecular Biology Reviews : MMBR·V Contamine, M Picard
Jan 14, 2000·Journal of Medical Genetics·M B DelatyckiS M Forrest
Jun 15, 2005·Annual Review of Biochemistry·Deborah C JohnsonMichael K Johnson
Dec 14, 1999·Biological Chemistry·E A CraigB Schilke
Jan 2, 2013·Frontiers in Bioscience (Landmark Edition)·Matthew G Baile, Steven M Claypool
Dec 18, 2001·Movement Disorders : Official Journal of the Movement Disorder Society·M Pandolfo
Feb 3, 2000·Proceedings of the National Academy of Sciences of the United States of America·H LangeR Lill
Jul 30, 2003·Proceedings of the National Academy of Sciences of the United States of America·Wing-Hang TongTracey A Rouault
Feb 15, 2001·Proceedings of the National Academy of Sciences of the United States of America·C VoisineE A Craig
Aug 2, 2003·Proceedings of the National Academy of Sciences of the United States of America·Edward LukValeria Cizewski Culotta
Jan 6, 2007·Biometals : an International Journal on the Role of Metal Ions in Biology, Biochemistry, and Medicine·Wing-Hang Tong, Tracey A Rouault
May 10, 2011·Brain Research Reviews·Daniele Marmolino
Jul 27, 2015·Mitochondrion·Shama Prasada KabekkoduKapaettu Satyamoorthy
Jul 9, 2008·Trends in Genetics : TIG·Tracey A Rouault, Wing Hang Tong
May 9, 2007·Mitochondrion·J M Cooper, A H V Schapira
Sep 24, 2014·Biochimica Et Biophysica Acta·Nunziata Maio, Tracey A Rouault

❮ Previous
Next ❯

Related Concepts

Related Feeds

ASBMB Publications

The American Society for Biochemistry and Molecular Biology (ASBMB) includes the Journal of Biological Chemistry, Molecular & Cellular Proteomics, and the Journal of Lipid Research. Discover the latest research from ASBMB here.

Ataxias

Ataxia is a neurological condition characterized by lack of voluntary coordination of muscle movements including loss of coordination, balance, and speech. Discover the latest research on different types of ataxias here.