Maturation of Frataxin Within Mammalian and Yeast Mitochondria: One-Step Processing by Matrix Processing Peptidase

Tricine-sodium dodecyl sulfate-polyacrylamide gel electrophoresis for the separation of proteins in the range from 1 to 100 kDa.

Schägger, H., & von Jagow, G. (1987) · Analytical Biochemistry · doi:10.1016/0003-2697(87)90587-2 · Cited by 11,420 · see citing works

Three-dimensional tracking of axonal projections in the brain by magnetic resonance imaging

Mori, S., Crain, B. J., Chacko, V. P., & van Zijl, P. C. M. (1999) · Annals of Neurology · doi:10.1002/1531-8249(199902)45:2 · Cited by 3,479 · see citing works

Friedreich's Ataxia: Autosomal Recessive Disease Caused by an Intronic GAA Triplet Repeat Expansion

Campuzano, V., Montermini, L., Moltò, M. D., Pianese, L., Cossée, M., Cavalcanti, F., Monros, E., Rodius, F., Duclos, F., Monticelli, A., et al. (1996) · Science · doi:10.1126/science.271.5254.1423 · Cited by 2,786 · see citing works

Functional messenger RNAs are produced by SP6 <i>in vitro</i> transcription of cloned cDNAs

Krieg, P. A., & Melton, D. A. (1984) · Nucleic Acids Research · doi:10.1093/nar/12.18.7057 · Cited by 1,533 · see citing works

The Biology of heat shock proteins and molecular chaperones.

Morimoto, R. I., Tissieres, A., & Georgopoulos, C. P. (1994) · Cited by 1,244 · see citing works

Clinical and Genetic Abnormalities in Patients with Friedreich's Ataxia

Dürr, A., Cossee, M., Agid, Y., Campuzano, V., Mignard, C., Penet, C., Mandel, J.-L., Brice, A., & Koenig, M. (1996) · New England Journal of Medicine · doi:10.1056/nejm199610173351601 · Cited by 1,071 · see citing works

PROTEIN IMPORT INTO MITOCHONDRIA

Neupert, W. (1997) · Annual Review of Biochemistry · doi:10.1146/annurev.biochem.66.1.863 · Cited by 1,060 · see citing works

Aconitase and mitochondrial iron–sulphur protein deficiency in Friedreich ataxia

Rötig, A., de Lonlay, P., Chretien, D., Foury, F., Koenig, M., Sidi, D., Munnich, A., & Rustin, P. (1997) · Nature Genetics · doi:10.1038/ng1097-215 · Cited by 991 · see citing works

[Protein import into mitochondria].

Zaĭtseva, L. G., Tv, O., & Grinkevich, V. A. (2000) · PubMed · Cited by 971 · see citing works

Regulation of Mitochondrial Iron Accumulation by Yfh1p, a Putative Homolog of Frataxin

Babcock, M., de Silva, D., Oaks, R., Davis-Kaplan, S., Jiralerspong, S., Montermini, L., Pandolfo, M., & Kaplan, J. (1997) · Science · doi:10.1126/science.276.5319.1709 · Cited by 921 · see citing works

American Journal of Human Genetics

(2005) · Encyclopedia of Statistical Sciences · doi:10.1002/0471667196.ess7082.pub2 · Cited by 874 · see citing works

Frataxin is Reduced in Friedreich Ataxia Patients and is Associated with Mitochondrial Membranes

Campuzano, V., Montermini, L., Lutz, Y., Cova, L., Hindelang, C., Jiralerspong, S., Trottier, Y., Kish, S. J., Faucheux, B. A., Trouillas, P., et al. (1997) · Human Molecular Genetics · doi:10.1093/hmg/6.11.1771 · Cited by 739 · see citing works

Studies of human, mouse and yeast homologues indicate a mitochondrial function for frataxin

Koutníková, H., Campuzano, V., Foury, F., Dollé, P., Cazzalini, O., & Koenig, M. (1997) · Nature Genetics · doi:10.1038/ng0897-345 · Cited by 473 · see citing works

Deletion of the yeast homologue of the human gene associated with Friedreich's ataxia elicits iron accumulation in mitochondria

Foury, F., & Cazzalini, O. (1997) · FEBS Letters · doi:10.1016/s0014-5793(97)00734-5 · Cited by 378 · see citing works

Respiratory deficiency due to loss of mitochondrial DNA in yeast lacking the frataxin homologue

Wilson, R. B., & Roof, D. M. (1997) · Nature Genetics · doi:10.1038/ng0897-352 · Cited by 345 · see citing works

The Yeast Frataxin Homologue Mediates Mitochondrial Iron Efflux

Radisky, D. C., Babcock, M. C., & Kaplan, J. (1999) · Journal of Biological Chemistry · doi:10.1074/jbc.274.8.4497 · Cited by 277 · see citing works

The ABC transporter Atm1p is required for mitochondrial iron homeostasis

Kispál, G., Csere, P., Guiard, B., & Lill, R. (1997) · FEBS Letters · doi:10.1016/s0014-5793(97)01414-2 · Cited by 276 · see citing works

An ABC transporter in the mitochondrial inner membrane is required for normal growth of yeast.

Leighton, J. A., & Schatz, G. (1995) · The EMBO Journal · doi:10.1002/j.1460-2075.1995.tb06989.x · Cited by 197 · see citing works

Suppressors of Superoxide Dismutase (SOD1) Deficiency in Saccharomyces cerevisiae

Strain, J., Lorenz, C. R., Bode, J., Garland, S., Smolen, G. A., Ta, D. T., Vickery, L. E., & Culotta, V. C. (1998) · Journal of Biological Chemistry · doi:10.1074/jbc.273.47.31138 · Cited by 181 · see citing works

Mt-Hsp70 Homolog, Ssc2p, Required for Maturation of Yeast Frataxin and Mitochondrial Iron Homeostasis

Knight, S. A. B., Sepuri, N. B. V., Pain, D., & Dancis, A. (1998) · Journal of Biological Chemistry · doi:10.1074/jbc.273.29.18389 · Cited by 173 · see citing works

Atypical Friedreich ataxia caused by compound heterozygosity for a novel missense mutation and the GAA triplet-repeat expansion.

Bidichandani, S. I., Ashizawa, T., & Patel, P. I. (1997) · Europe PMC (PubMed Central) · Cited by 141 · see citing works

The mitochondrial targeting presequence of the Rieske iron-sulfur protein is processed in a single step after insertion into the cytochrome bc1 complex in mammals and retained as a subunit in the complex.

Brandt, U., Yu, L. P. C., Yu, C. A., et al. (1993) · Journal of Biological Chemistry · doi:10.1016/s0021-9258(18)52883-0 · Cited by 127 · see citing works

Maturation of wild-type and mutated frataxin by the mitochondrial processing peptidase

Koutníková, H. (1998) · Human Molecular Genetics · doi:10.1093/hmg/7.9.1485 · Cited by 122 · see citing works

Pathology of the Heart in Friedreich's Ataxia: Review of the Literature and Report of One Case

Sanchez-Casis, G., Cote, M., & Barbeau, A. (1976) · Canadian Journal of Neurological Sciences / Journal Canadien des Sciences Neurologiques · doi:10.1017/s0317167100025580 · Cited by 103 · see citing works

Identification of the human mitochondrial protein import receptor, huMas20p. Complementation of Δmas20 in yeast

Goping, I. S., Millar, D. G., & Shore, G. C. (1995) · FEBS Letters · doi:10.1016/0014-5793(95)01010-c · Cited by 83 · see citing works

The Preprotein Translocase of the Inner Mitochondrial Membrane: Evolutionary Conservation of Targeting and Assembly of Tim17

Bömer, U., Rassow, J., Zufall, N., et al. (1996) · Journal of Molecular Biology · doi:10.1006/jmbi.1996.0522 · Cited by 63 · see citing works

Functional cooperation of the mitochondrial processing peptidase subunits

Luciano, P., Geoffroy, S., Brandt, A., Hernandez, J., & Géli, V. (1997) · Journal of Molecular Biology · doi:10.1006/jmbi.1997.1231 · Cited by 61 · see citing works

In vivo zippering of inner and outer mitochondrial membranes by a stable translocation intermediate

Schülke, N., Sepuri, N. B. V., & Pain, D. (1997) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.94.14.7314 · Cited by 50 · see citing works

Import of the iron-sulfur protein of the cytochrome b.c1 complex into yeast mitochondria.

Fu, W., Japa, S., & Beattie, D. S. (1990) · Journal of Biological Chemistry · doi:10.1016/s0021-9258(17)46257-0 · Cited by 49 · see citing works

Physiological Role of the N-terminal Processed P4501A1 Targeted to Mitochondria in Erythromycin Metabolism and Reversal of Erythromycin-mediated Inhibition of Mitochondrial Protein Synthesis

Anandatheerthavarada, H. K., Vijayasarathy, C., Bhagwat, S. V., Biswas, G., Mullick, J., & Avadhani, N. G. (1999) · Journal of Biological Chemistry · doi:10.1074/jbc.274.10.6617 · Cited by 47 · see citing works

The Yeast Connection to Friedreich Ataxia

Knight, S. A. B., Kim, R., Pain, D., & Dancis, A. (1999) · The American Journal of Human Genetics · doi:10.1086/302270 · Cited by 42 · see citing works

Functional reconstitution in Escherichia coli of the yeast mitochondrial matrix peptidase from its two inactive subunits.

Géli, V. (1993) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.90.13.6247 · Cited by 39 · see citing works

Mutational Analysis of Both Subunits from Rat Mitochondrial Processing Peptidase

Striebel, H., Ryšavý, P., Adamec, J., et al. (1996) · Archives of Biochemistry and Biophysics · doi:10.1006/abbi.1996.0500 · Cited by 37 · see citing works

Import and processing of human ornithine transcarbamoylase precursor by mitochondria from Saccharomyces cerevisiae.

Cheng, M. Y., Pollock, R. A., Hendrick, J. P., et al. (1987) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.84.12.4063 · Cited by 35 · see citing works

GTP Hydrolysis Is Essential for Protein Import into the Mitochondrial Matrix

Sepuri, N. B. V., Schülke, N., & Pain, D. (1998) · Journal of Biological Chemistry · doi:10.1074/jbc.273.3.1420 · Cited by 35 · see citing works

A GTP-dependent “Push” Is Generally Required for Efficient Protein Translocation across the Mitochondrial Inner Membrane into the Matrix

Sepuri, N. B. V., Gordon, D. M., & Pain, D. (1998) · Journal of Biological Chemistry · doi:10.1074/jbc.273.33.20941 · Cited by 29 · see citing works

Two-step Processing Is Not Essential for the Import and Assembly of Functionally Active Iron-Sulfur Protein into the Cytochrome bc1 Complex in Saccharomyces cerevisiae

Nett, J. H., Denke, E., & Trumpower, B. L. (1997) · Journal of Biological Chemistry · doi:10.1074/jbc.272.4.2212 · Cited by 24 · see citing works

Processing of the Presequence of the Schizosaccharomyces pombe Rieske Iron-Sulfur Protein Occurs in a Single Step and Can Be Converted to Two-step Processing by Mutation of a Single Proline to Serine in the Presequence

Nett, J. H., Schägger, H., & Trumpower, B. L. (1998) · Journal of Biological Chemistry · doi:10.1074/jbc.273.15.8652 · Cited by 14 · see citing works

Intracellular iron transport and storage: from molecular mechanisms to health implications

Mackenzie, E. L., Iwasaki, K., & Tsuji, Y. (2008) · Antioxidants &amp; Redox Signaling · doi:10.1089/ars.2007.1893 · Cited by 526 · see citing works

Crystal Structure of Human Frataxin

Dhe-Paganon, S., Shigeta, R., Chi, Y.-I., Ristow, M., & Shoelson, S. E. (2000) · Journal of Biological Chemistry · doi:10.1074/jbc.c000407200 · Cited by 226 · see citing works

Towards a structural understanding of Friedreich’s ataxia: the solution structure of frataxin

Musco, G., Stier, G., Kolmerer, B., Adinolfi, S., Martin, S., Frenkiel, T., Gibson, T., & Pastore, A. (2000) · Structure · doi:10.1016/s0969-2126(00)00158-1 · Cited by 166 · see citing works

Friedreich Ataxia: From GAA Triplet–Repeat Expansion to Frataxin Deficiency

Patel, P. I., & Isaya, G. (2001) · The American Journal of Human Genetics · doi:10.1086/321283 · Cited by 126 · see citing works

In vivo maturation of human frataxin

Condò, I., Ventura, N., Malisan, F., Rufini, A., Tomassini, B., & Testi, R. (2007) · Human Molecular Genetics · doi:10.1093/hmg/ddm102 · Cited by 118 · see citing works

Advancements in the pathophysiology of Friedreich’s Ataxia and new prospects for treatments

Babady, N. E., Carelle, N., Wells, R. D., Rouault, T. A., Hirano, M., Lynch, D. R., Delatycki, M. B., Wilson, R. B., Isaya, G., & Puccio, H. (2007) · Molecular Genetics and Metabolism · doi:10.1016/j.ymgme.2007.05.009 · Cited by 73 · see citing works

Oligomerization of human and bacterial frataxin : Structural and functional studies

Abdalkhalik, M. (2017) · Lund University Publications (Lund University)

A structural approach to understanding the iron-binding properties of phylogenetically different frataxins

Adinolfi, S. (2002) · Human Molecular Genetics · doi:10.1093/hmg/11.16.1865 · Cited by 133 · see citing works

The yeast frataxin homolog Yfh1p plays a specific role in the maturation of cellular Fe/S proteins

Mühlenhoff, U. (2002) · Human Molecular Genetics · doi:10.1093/hmg/11.17.2025 · Cited by 326 · see citing works

The expression of human mitochondrial ferritin rescues respiratory function infrataxin-deficient yeast

Campanella, A. (2004) · Human Molecular Genetics · doi:10.1093/hmg/ddh232 · Cited by 109 · see citing works

Towards a metabolomic approach to investigate iron–sulfur cluster biogenesis

Marengo, M., Fissore, A., Oliaro‐Bosso, S., Adinolfi, S., & Pastore, A. (2022) · IUBMB Life · doi:10.1002/iub.2618 · Cited by 4 · see citing works