| Year |
Citation |
Score |
| 2017 |
Ahlgren EC, Fekry M, Wiemann M, Söderberg CA, Bernfur K, Gakh O, Rasmussen M, Højrup P, Emanuelsson C, Isaya G, Al-Karadaghi S. Iron-induced oligomerization of human FXN81-210 and bacterial CyaY frataxin and the effect of iron chelators. Plos One. 12: e0188937. PMID 29200434 DOI: 10.1371/Journal.Pone.0188937 |
0.502 |
|
| 2017 |
Fekry M, Alshokry W, Grela P, Tchórzewski M, Ahlgren EC, Söderberg CA, Gakh O, Isaya G, Al-Karadaghi S. SAXS and stability studies of iron-induced oligomers of bacterial frataxin CyaY. Plos One. 12: e0184961. PMID 28931050 DOI: 10.1371/Journal.Pone.0184961 |
0.508 |
|
| 2017 |
Gakh O, Ranatunga W, Galeano BK, Smith DS, Thompson JR, Isaya G. Defining the Architecture of the Core Machinery for the Assembly of Fe-S Clusters in Human Mitochondria. Methods in Enzymology. 595: 107-160. PMID 28882199 DOI: 10.1016/Bs.Mie.2017.07.003 |
0.447 |
|
| 2017 |
Galeano BK, Ranatunga W, Gakh O, Smith DY, Thompson JR, Isaya G. Zinc and the iron donor frataxin regulate oligomerization of the scaffold protein to form new Fe-S cluster assembly centers. Metallomics : Integrated Biometal Science. PMID 28548666 DOI: 10.1039/C7Mt00089H |
0.49 |
|
| 2016 |
Gakh O, Ranatunga W, Smith DY, Ahlgren EC, Al-Karadaghi S, Thompson JR, Isaya G. Architecture of the Human Mitochondrial Iron-Sulfur Cluster Assembly Machinery. The Journal of Biological Chemistry. PMID 27519411 DOI: 10.1074/Jbc.M116.738542 |
0.472 |
|
| 2016 |
Soderberg CG, Gillam ME, Ahlgren EC, Hunter GA, Gakh O, Isaya G, Ferreira GC, Al-Karadaghi S. The Structure of the Complex Between Yeast Frataxin and Ferrochelatase: Characterization and pre-Steady State Reaction of Ferrous Iron Delivery and Heme Synthesis. The Journal of Biological Chemistry. PMID 27026703 DOI: 10.1074/Jbc.M115.701128 |
0.507 |
|
| 2016 |
Ranatunga W, Gakh O, Galeano BK, Smith DY, Söderberg CA, Al-Karadaghi S, Thompson JR, Isaya G. Architecture of the Yeast Mitochondrial Iron-Sulfur Cluster Assembly Machinery: The Sub-Complex Formed by the Iron Donor, Yfh1, and the Scaffold, Isu1. The Journal of Biological Chemistry. PMID 26941001 DOI: 10.1074/Jbc.M115.712414 |
0.502 |
|
| 2015 |
Yoon G, Delague V, Mégarbané A, Isaya G. Reply: Autosomal recessive cerebellar ataxia caused by a homozygous mutation in PMPCA. Brain : a Journal of Neurology. PMID 26657516 DOI: 10.1093/Brain/Awv363 |
0.352 |
|
| 2015 |
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, ... ... Isaya G, et al. PMPCA mutations cause abnormal mitochondrial protein processing in patients with non-progressive cerebellar ataxia. Brain : a Journal of Neurology. 138: 1505-17. PMID 25808372 DOI: 10.1093/Brain/Awv057 |
0.442 |
|
| 2014 |
Isaya G. Mitochondrial iron-sulfur cluster dysfunction in neurodegenerative disease. Frontiers in Pharmacology. 5: 29. PMID 24624085 DOI: 10.3389/Fphar.2014.00029 |
0.532 |
|
| 2013 |
Söderberg CA, Rajan S, Shkumatov AV, Gakh O, Schaefer S, Ahlgren EC, Svergun DI, Isaya G, Al-Karadaghi S. The molecular basis of iron-induced oligomerization of frataxin and the role of the ferroxidation reaction in oligomerization. The Journal of Biological Chemistry. 288: 8156-67. PMID 23344952 DOI: 10.1074/Jbc.M112.442285 |
0.531 |
|
| 2013 |
Li H, Gakh O, Smith DY, Ranatunga WK, Isaya G. Missense mutations linked to friedreich ataxia have different but synergistic effects on mitochondrial frataxin isoforms. The Journal of Biological Chemistry. 288: 4116-27. PMID 23269675 DOI: 10.1074/Jbc.M112.435263 |
0.593 |
|
| 2013 |
Vaubel RA, Isaya G. Iron-sulfur cluster synthesis, iron homeostasis and oxidative stress in Friedreich ataxia. Molecular and Cellular Neurosciences. 55: 50-61. PMID 22917739 DOI: 10.1016/J.Mcn.2012.08.003 |
0.718 |
|
| 2011 |
Söderberg CA, Shkumatov AV, Rajan S, Gakh O, Svergun DI, Isaya G, Al-Karadaghi S. Oligomerization propensity and flexibility of yeast frataxin studied by X-ray crystallography and small-angle X-ray scattering. Journal of Molecular Biology. 414: 783-97. PMID 22051511 DOI: 10.1016/J.Jmb.2011.10.034 |
0.368 |
|
| 2011 |
Vaubel RA, Rustin P, Isaya G. Mutations in the dimer interface of dihydrolipoamide dehydrogenase promote site-specific oxidative damages in yeast and human cells. The Journal of Biological Chemistry. 286: 40232-45. PMID 21930696 DOI: 10.1074/Jbc.M111.274415 |
0.657 |
|
| 2011 |
Bedekovics T, Li H, Gajdos GB, Isaya G. Leucine biosynthesis regulates cytoplasmic iron-sulfur enzyme biogenesis in an Atm1p-independent manner. The Journal of Biological Chemistry. 286: 40878-88. PMID 21926174 DOI: 10.1074/Jbc.M111.270082 |
0.543 |
|
| 2011 |
Gakh O, Bedekovics T, Duncan SF, Smith IV DY, Berkholz DS, Isaya G. Normal and Friedreich ataxia cells express different isoforms of frataxin with complementary roles in iron-sulfur cluster assembly (Journal of Biological Chemistry (2010) 285, (38486-38501)) Journal of Biological Chemistry. 286: 8708. DOI: 10.1074/Jbc.A110.145144 |
0.418 |
|
| 2010 |
Gakh O, Bedekovics T, Duncan SF, Smith DY, Berkholz DS, Isaya G. Normal and Friedreich ataxia cells express different isoforms of frataxin with complementary roles in iron-sulfur cluster assembly. The Journal of Biological Chemistry. 285: 38486-501. PMID 20889968 DOI: 10.1074/Jbc.M110.145144 |
0.528 |
|
| 2009 |
Li H, Gakh O, Smith DY, Isaya G. Oligomeric yeast frataxin drives assembly of core machinery for mitochondrial iron-sulfur cluster synthesis. The Journal of Biological Chemistry. 284: 21971-80. PMID 19491103 DOI: 10.1074/Jbc.M109.011197 |
0.547 |
|
| 2008 |
Gakh O, Smith DY, Isaya G. Assembly of the iron-binding protein frataxin in Saccharomyces cerevisiae responds to dynamic changes in mitochondrial iron influx and stress level. The Journal of Biological Chemistry. 283: 31500-10. PMID 18784075 DOI: 10.1074/Jbc.M805415200 |
0.592 |
|
| 2008 |
Willis JH, Isaya G, Gakh O, Capaldi RA, Marusich MF. Lateral-flow immunoassay for the frataxin protein in Friedreich's ataxia patients and carriers. Molecular Genetics and Metabolism. 94: 491-7. PMID 18485778 DOI: 10.1016/J.Ymgme.2008.03.019 |
0.311 |
|
| 2008 |
Schagerlöf U, Elmlund H, Gakh O, Nordlund G, Hebert H, Lindahl M, Isaya G, Al-Karadaghi S. Structural basis of the iron storage function of frataxin from single-particle reconstruction of the iron-loaded oligomer. Biochemistry. 47: 4948-54. PMID 18393441 DOI: 10.1021/Bi800052M |
0.463 |
|
| 2007 |
Bedekovics T, Gajdos GB, Kispal G, Isaya G. Partial conservation of functions between eukaryotic frataxin and the Escherichia coli frataxin homolog CyaY. Fems Yeast Research. 7: 1276-84. PMID 17727661 DOI: 10.1111/J.1567-1364.2007.00296.X |
0.578 |
|
| 2007 |
Babady NE, Pang YP, Elpeleg O, Isaya G. Cryptic proteolytic activity of dihydrolipoamide dehydrogenase. Proceedings of the National Academy of Sciences of the United States of America. 104: 6158-63. PMID 17404228 DOI: 10.1073/Pnas.0610618104 |
0.404 |
|
| 2007 |
Isaya G. Ironing out a therapy for Friedreich ataxia Blood. 110: 1-2. DOI: 10.1182/Blood-2007-04-084509 |
0.498 |
|
| 2006 |
Karlberg T, Schagerlöf U, Gakh O, Park S, Ryde U, Lindahl M, Leath K, Garman E, Isaya G, Al-Karadaghi S. The structures of frataxin oligomers reveal the mechanism for the delivery and detoxification of iron. Structure (London, England : 1993). 14: 1535-46. PMID 17027502 DOI: 10.1016/J.Str.2006.08.010 |
0.491 |
|
| 2006 |
Al-Karadaghi S, Franco R, Hansson M, Shelnutt JA, Isaya G, Ferreira GC. Chelatases: distort to select? Trends in Biochemical Sciences. 31: 135-42. PMID 16469498 DOI: 10.1016/J.Tibs.2006.01.001 |
0.312 |
|
| 2006 |
Gakh O, Park S, Liu G, Macomber L, Imlay JA, Ferreira GC, Isaya G. Mitochondrial iron detoxification is a primary function of frataxin that limits oxidative damage and preserves cell longevity. Human Molecular Genetics. 15: 467-79. PMID 16371422 DOI: 10.1093/Hmg/Ddi461 |
0.566 |
|
| 2005 |
Ensenauer R, He M, Willard JM, Goetzman ES, Corydon TJ, Vandahl BB, Mohsen AW, Isaya G, Vockley J. Human acyl-CoA dehydrogenase-9 plays a novel role in the mitochondrial beta-oxidation of unsaturated fatty acids. The Journal of Biological Chemistry. 280: 32309-16. PMID 16020546 DOI: 10.1074/Jbc.M504460200 |
0.402 |
|
| 2005 |
Van Driest SL, Gakh O, Ommen SR, Isaya G, Ackerman MJ. Molecular and functional characterization of a human frataxin mutation found in hypertrophic cardiomyopathy. Molecular Genetics and Metabolism. 85: 280-5. PMID 15936968 DOI: 10.1016/J.Ymgme.2005.04.010 |
0.407 |
|
| 2005 |
Bulteau AL, Lundberg KC, Ikeda-Saito M, Isaya G, Szweda LI. Reversible redox-dependent modulation of mitochondrial aconitase and proteolytic activity during in vivo cardiac ischemia/reperfusion. Proceedings of the National Academy of Sciences of the United States of America. 102: 5987-91. PMID 15840721 DOI: 10.1073/Pnas.0501519102 |
0.451 |
|
| 2005 |
O'Neill HA, Gakh O, Park S, Cui J, Mooney SM, Sampson M, Ferreira GC, Isaya G. Assembly of human frataxin is a mechanism for detoxifying redox-active iron. Biochemistry. 44: 537-45. PMID 15641778 DOI: 10.1021/Bi048459J |
0.568 |
|
| 2005 |
O'Neill HA, Gakh O, Isaya G. Supramolecular assemblies of human frataxin are formed via subunit-subunit interactions mediated by a non-conserved amino-terminal region. Journal of Molecular Biology. 345: 433-9. PMID 15581888 DOI: 10.1016/J.Jmb.2004.10.074 |
0.494 |
|
| 2004 |
Campanella A, Isaya G, O'Neill HA, Santambrogio P, Cozzi A, Arosio P, Levi S. The expression of human mitochondrial ferritin rescues respiratory function in frataxin-deficient yeast. Human Molecular Genetics. 13: 2279-88. PMID 15282205 DOI: 10.1093/Hmg/Ddh232 |
0.586 |
|
| 2004 |
Bulteau AL, O'Neill HA, Kennedy MC, Ikeda-Saito M, Isaya G, Szweda LI. Frataxin acts as an iron chaperone protein to modulate mitochondrial aconitase activity. Science (New York, N.Y.). 305: 242-5. PMID 15247478 DOI: 10.1126/Science.1098991 |
0.586 |
|
| 2004 |
Isaya G, O'Neill HA, Gakh O, Park S, Mantcheva R, Mooney SM. Functional studies of frataxin. Acta Paediatrica (Oslo, Norway : 1992). Supplement. 93: 68-71; discussion 72. PMID 15176725 DOI: 10.1111/J.1651-2227.2004.Tb03061.X |
0.565 |
|
| 2004 |
Isaya G. Mitochondrial intermediate peptidase Handbook of Proteolytic Enzymes: Second Edition. 1: 366-369. DOI: 10.1016/0076-6879(95)48035-8 |
0.457 |
|
| 2003 |
Karthikeyan G, Santos JH, Graziewicz MA, Copeland WC, Isaya G, Van Houten B, Resnick MA. Reduction in frataxin causes progressive accumulation of mitochondrial damage. Human Molecular Genetics. 12: 3331-42. PMID 14570713 DOI: 10.1093/Hmg/Ddg349 |
0.495 |
|
| 2003 |
Nichol H, Gakh O, O'Neill HA, Pickering IJ, Isaya G, George GN. Structure of frataxin iron cores: an X-ray absorption spectroscopic study. Biochemistry. 42: 5971-6. PMID 12755598 DOI: 10.1021/Bi027021L |
0.435 |
|
| 2003 |
Park S, Gakh O, O'Neill HA, Mangravita A, Nichol H, Ferreira GC, Isaya G. Yeast frataxin sequentially chaperones and stores iron by coupling protein assembly with iron oxidation. The Journal of Biological Chemistry. 278: 31340-51. PMID 12732649 DOI: 10.1074/Jbc.M303158200 |
0.529 |
|
| 2002 |
Gakh O, Cavadini P, Isaya G. Mitochondrial processing peptidases. Biochimica Et Biophysica Acta. 1592: 63-77. PMID 12191769 DOI: 10.1016/S0167-4889(02)00265-3 |
0.437 |
|
| 2002 |
Park S, Gakh O, Mooney SM, Isaya G. The ferroxidase activity of yeast frataxin. The Journal of Biological Chemistry. 277: 38589-95. PMID 12149269 DOI: 10.1074/Jbc.M206711200 |
0.398 |
|
| 2002 |
Cavadini P, Gakh O, Isaya G. Protein import and processing reconstituted with isolated rat liver mitochondria and recombinant mitochondrial processing peptidase. Methods (San Diego, Calif.). 26: 298-306. PMID 12054920 DOI: 10.1016/S1046-2023(02)00035-X |
0.451 |
|
| 2002 |
Gakh O, Adamec J, Gacy AM, Twesten RD, Owen WG, Isaya G. Physical evidence that yeast frataxin is an iron storage protein. Biochemistry. 41: 6798-804. PMID 12022884 DOI: 10.1021/Bi025566+ |
0.542 |
|
| 2002 |
Cavadini P, O'Neill HA, Benada O, Isaya G. Assembly and iron-binding properties of human frataxin, the protein deficient in Friedreich ataxia. Human Molecular Genetics. 11: 217-27. PMID 11823441 |
0.521 |
|
| 2002 |
Adamec J, Kalousek F, Isaya G. 4 Mitochondrial processing peptidase/mitochondrial intermediate peptidase Enzymes. 22: 77-100. DOI: 10.1016/S1874-6047(02)80005-1 |
0.408 |
|
| 2001 |
Patel PI, Isaya G. Friedreich ataxia: From GAA triplet-repeat expansion to frataxin deficiency American Journal of Human Genetics. 69: 15-24. PMID 11391483 DOI: 10.1086/321283 |
0.423 |
|
| 2000 |
Cavadini P, Gellera C, Patel PI, Isaya G. Human frataxin maintains mitochondrial iron homeostasis in Saccharomyces cerevisiae Human Molecular Genetics. 9: 2523-2530. PMID 11030757 DOI: 10.1093/Hmg/9.17.2523 |
0.611 |
|
| 2000 |
Cavadini P, Adamec J, Taroni F, Gakh O, Isaya G. Two-step processing of human frataxin by mitochondrial processing peptidase: Precursor and intermediate forms are cleaved at different rates Journal of Biological Chemistry. 275: 41469-41475. PMID 11020385 DOI: 10.1074/Jbc.M006539200 |
0.307 |
|
| 2000 |
Adamec J, Rusnak F, Owen WG, Naylor S, Benson LM, Gacy AM, Isaya G. Iron-dependent self-assembly of recombinant yeast frataxin: implications for Friedreich ataxia. American Journal of Human Genetics. 67: 549-62. PMID 10930361 DOI: 10.1086/303056 |
0.486 |
|
| 2000 |
Chew A, Sirugo G, Alsobrook JP, Isaya G. Functional and genomic analysis of the human mitochondrial intermediate peptidase, a putative protein partner of frataxin. Genomics. 65: 104-12. PMID 10783257 DOI: 10.1006/Geno.2000.6162 |
0.537 |
|
| 1999 |
Branda SS, Cavadini P, Adamec J, Kalousek F, Taroni F, Isaya G. Yeast and human frataxin are processed to mature form in two sequential steps by the mitochondrial processing peptidase Journal of Biological Chemistry. 274: 22763-22769. PMID 10428860 DOI: 10.1074/Jbc.274.32.22763 |
0.574 |
|
| 1999 |
Branda SS, Yang ZY, Chew A, Isaya G. Mitochondrial intermediate peptidase and the yeast frataxin homolog together maintain mitochondrial iron homeostasis in Saccharomyces cerevisiae. Human Molecular Genetics. 8: 1099-110. PMID 10332043 DOI: 10.1093/Hmg/8.6.1099 |
0.593 |
|
| 1999 |
Ashmarina LI, Pshezhetsky AV, Branda SS, Isaya G, Mitchell GA. 3-Hydroxy-3-methylglutaryl coenzyme A lyase: Targeting and processing in peroxisomes and mitochondria Journal of Lipid Research. 40: 70-75. PMID 9869651 |
0.3 |
|
| 1997 |
Chew A, Buck EA, Peretz S, Sirugo G, Rinaldo P, Isaya G. Cloning, expression, and chromosomal assignment of the human mitochondrial intermediate peptidase gene (MIPEP). Genomics. 40: 493-6. PMID 9073519 DOI: 10.1006/Geno.1996.4586 |
0.442 |
|
| 1996 |
Chew A, Rollins RA, Sakati WR, Isaya G. Mutations in a putative zinc-binding domain inactivate the mitochondrial intermediate peptidase. Biochemical and Biophysical Research Communications. 226: 822-9. PMID 8831696 DOI: 10.1006/Bbrc.1996.1435 |
0.343 |
|
| 1995 |
Goodman SI, Kratz LE, DiGiulio KA, Biery BJ, Goodman KE, Isaya G, Frerman FE. Cloning of glutaryl-CoA dehydrogenase cDNA, and expression of wild type and mutant enzymes in Escherichia coli. Human Molecular Genetics. 4: 1493-8. PMID 8541831 DOI: 10.1093/Hmg/4.9.1493 |
0.363 |
|
| 1995 |
Branda SS, Isaya G. Prediction and identification of new natural substrates of the yeast mitochondrial intermediate peptidase Journal of Biological Chemistry. 270: 27366-27373. PMID 7593000 DOI: 10.1074/Jbc.270.45.27366 |
0.471 |
|
| 1995 |
Isaya G, Sakati WR, Rollins RA, Shen GP, Hanson LQ, Ullrich RC, Novotny CP. Mammalian Mitochondrial Intermediate Peptidase: Structure/Function Analysis of a New Homologue from Schizophyllum commune and Relationship to Thimet Oligopeptidases Genomics. 28: 450-461. PMID 7490080 DOI: 10.1006/Geno.1995.1174 |
0.426 |
|
| 1994 |
Isaya G, Miklos D, Rollins RA. MIP1, a new yeast gene homologous to the rat mitochondrial intermediate peptidase gene, is required for oxidative metabolism in Saccharomyces cerevisiae Molecular and Cellular Biology. 14: 5603-5616. PMID 8035833 DOI: 10.1128/Mcb.14.8.5603 |
0.52 |
|
| 1992 |
Isaya G, Kalousek F, Rosenberg LE. Amino-terminal octapeptides function as recognition signals for the mitochondrial intermediate peptidase Journal of Biological Chemistry. 267: 7904-7910. PMID 1560019 |
0.316 |
|
| 1992 |
Isaya G, Kalousek F, Rosenberg LE. Sequence analysis of rat mitochondrial intermediate peptidase: Similarity to zinc metallopeptidases and to a putative yeast homologue Proceedings of the National Academy of Sciences of the United States of America. 89: 8317-8321. PMID 1518864 DOI: 10.1073/Pnas.89.17.8317 |
0.41 |
|
| 1992 |
Kalousek F, Isaya G, Rosenberg LE. Rat liver mitochondrial intermediate peptidase (MIP): Purification and initial characterization Embo Journal. 11: 2803-2809. PMID 1322290 DOI: 10.1002/J.1460-2075.1992.Tb05347.X |
0.46 |
|
| 1991 |
Purdue PE, Allsop J, Isaya G, Rosenberg LE, Danpure CJ. Mistargeting of peroxisomal L-alanine:glyoxylate aminotransferase to mitochondria in primary hyperoxaluria patients depends upon activation of a cryptic mitochondrial targeting sequence by a point mutation Proceedings of the National Academy of Sciences of the United States of America. 88: 10900-10904. PMID 1961759 DOI: 10.1073/Pnas.88.23.10900 |
0.448 |
|
| 1991 |
Isaya G, Kalousek F, Fenton WA, Rosenberg LE. Cleavage of precursors by the mitochondrial processing peptidase requires a compatible mature protein or an intermediate octapeptide Journal of Cell Biology. 113: 65-76. PMID 1672532 DOI: 10.1083/Jcb.113.1.65 |
0.435 |
|
| 1988 |
Isaya G, Fenton WA, Hendrick JP, Furtak K, Kalousek F, Rosenberg LE. Mitochondrial import and processing of mutant human ornithine transcarbamylase precursors in cultured cells Molecular and Cellular Biology. 8: 5150-5158. PMID 3244350 DOI: 10.1128/Mcb.8.12.5150 |
0.449 |
|
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