-
1
-
-
0019521898
-
Early onset cerebellar ataxia with retained tendon reflexes: A clinical and genetic study of a disorder distinct from Friedreich's ataxia
-
Harding, A.E. (1981) Early onset cerebellar ataxia with retained tendon reflexes: A clinical and genetic study of a disorder distinct from Friedreich's ataxia. J. Neurol. Neurosurg. Psychiatry, 44, 503-508.
-
(1981)
J. Neurol. Neurosurg. Psychiatry
, vol.44
, pp. 503-508
-
-
Harding, A.E.1
-
2
-
-
0021067564
-
The heart disease of Friedreich's ataxia: A clinical and electrocardiographic study of 115 patients, with an analysis of serial electrocardiographic changes in 30 cases
-
Harding, A.E. and Hewer, R.L. (1983) The heart disease of Friedreich's ataxia: A clinical and electrocardiographic study of 115 patients, with an analysis of serial electrocardiographic changes in 30 cases. Q. J. Med., 52, 489-502.
-
(1983)
Q. J. Med
, vol.52
, pp. 489-502
-
-
Harding, A.E.1
Hewer, R.L.2
-
3
-
-
0023802480
-
Glucose metabolism alterations in Friedreich's ataxia
-
Finocchiaro, G., Baio, G., Micossi, P., Pozza, G. and di Donato, S. (1988) Glucose metabolism alterations in Friedreich's ataxia. Neurology, 38, 1292-1296.
-
(1988)
Neurology
, vol.38
, pp. 1292-1296
-
-
Finocchiaro, G.1
Baio, G.2
Micossi, P.3
Pozza, G.4
di Donato, S.5
-
4
-
-
13344270899
-
Friedreich's ataxia: Autosomal recessive disease caused by an intronic GAA triplet repeat expansion
-
Campuzano, V., Montermini, L., Molto, M.D., Pianese, L., Cossee, M., Cavalcanti, F., Monros, E., Rodius, F., Duclos, F., Monticelli, A. et al. (1996) Friedreich's ataxia: Autosomal recessive disease caused by an intronic GAA triplet repeat expansion. Science, 271, 1423-1427.
-
(1996)
Science
, vol.271
, pp. 1423-1427
-
-
Campuzano, V.1
Montermini, L.2
Molto, M.D.3
Pianese, L.4
Cossee, M.5
Cavalcanti, F.6
Monros, E.7
Rodius, F.8
Duclos, F.9
Monticelli, A.10
-
5
-
-
0033120042
-
Sticky DNA: Self-association properties of long GAA.TTC repeats in R.R.Y triplex structures from Friedreich's ataxia
-
Sakamoto, N., Chastain, P.D., Parniewski, P. and Ohshima, K. (1999) Sticky DNA: Self-association properties of long GAA.TTC repeats in R.R.Y triplex structures from Friedreich's ataxia. Mol. Cell, 3, 465-475.
-
(1999)
Mol. Cell
, vol.3
, pp. 465-475
-
-
Sakamoto, N.1
Chastain, P.D.2
Parniewski, P.3
Ohshima, K.4
-
6
-
-
33746795976
-
DNA sequence-specific polyamides alleviate transcription inhibition associated with long GAA. TTC repeats in Friedreich's ataxia
-
Burnett, R., Melander, C., Puckett, J.W., Son, L.S., Wells, R.D., Dervan, P.B. and Gottesfeld, J.M. (2006) DNA sequence-specific polyamides alleviate transcription inhibition associated with long GAA. TTC repeats in Friedreich's ataxia. Proc. Natl. Acad. Sci. USA, 103, 11497-11502.
-
(2006)
Proc. Natl. Acad. Sci. USA
, vol.103
, pp. 11497-11502
-
-
Burnett, R.1
Melander, C.2
Puckett, J.W.3
Son, L.S.4
Wells, R.D.5
Dervan, P.B.6
Gottesfeld, J.M.7
-
7
-
-
33748778745
-
Histone deacetylase inhibitors reverse gene silencing in Friedreich's ataxia
-
Herman, D., Jenssen, K., Burnett, R., Soragni, E., Perlman, S.L. and Gottesfeld, J.M. (2006) Histone deacetylase inhibitors reverse gene silencing in Friedreich's ataxia. Nat. Chem. Biol., 2, 551-558.
-
(2006)
Nat. Chem. Biol
, vol.2
, pp. 551-558
-
-
Herman, D.1
Jenssen, K.2
Burnett, R.3
Soragni, E.4
Perlman, S.L.5
Gottesfeld, J.M.6
-
8
-
-
0031941447
-
The GAA triplet-repeat expansion in Friedreich ataxia interferes with transcription and may be associated with an unusual DNA structure
-
Bidichandani, S.I., Ashizawa, T. and Patel, P.I. (1998) The GAA triplet-repeat expansion in Friedreich ataxia interferes with transcription and may be associated with an unusual DNA structure. Am. J. Hum. Genet., 62, 111-121.
-
(1998)
Am. J. Hum. Genet
, vol.62
, pp. 111-121
-
-
Bidichandani, S.I.1
Ashizawa, T.2
Patel, P.I.3
-
9
-
-
0030815628
-
Somatic mosaicism for Friedreich's ataxia GAA triplet repeat expansions in the central nervous system
-
Montermini, L., Kish, S.J., Jiralerspong, S., Lamarche, J.B. and Pandolfo, M. (1997) Somatic mosaicism for Friedreich's ataxia GAA triplet repeat expansions in the central nervous system. Neurology 49, 606-610.
-
(1997)
Neurology
, vol.49
, pp. 606-610
-
-
Montermini, L.1
Kish, S.J.2
Jiralerspong, S.3
Lamarche, J.B.4
Pandolfo, M.5
-
10
-
-
0032800601
-
Low iron concentration and aconitase deficiency in a yeast frataxin homologue deficient strain
-
Foury, F. (1999) Low iron concentration and aconitase deficiency in a yeast frataxin homologue deficient strain. FEBS Lett., 456, 281-284.
-
(1999)
FEBS Lett
, vol.456
, pp. 281-284
-
-
Foury, F.1
-
11
-
-
0037613459
-
iron-sulfur cluster biosynthesis. Characterization of frataxin as an iron donor for assembly of [2Fe-2S] clusters in ISU-type proteins
-
Yoon, T. and Cowan, J.A. (2003) iron-sulfur cluster biosynthesis. Characterization of frataxin as an iron donor for assembly of [2Fe-2S] clusters in ISU-type proteins. J. Am. Chem. Soc., 125, 6078-6094.
-
(2003)
J. Am. Chem. Soc
, vol.125
, pp. 6078-6094
-
-
Yoon, T.1
Cowan, J.A.2
-
12
-
-
0141623560
-
An interaction between frataxin and Isu1/Nfs1 that is crucial for Fe/S cluster synthesis on Isu1
-
Gerber, J. Muhlenhoff, U. and Lill, R. (2003) An interaction between frataxin and Isu1/Nfs1 that is crucial for Fe/S cluster synthesis on Isu1. EMBO Rep., 4, 906-911.
-
(2003)
EMBO Rep
, vol.4
, pp. 906-911
-
-
Gerber, J.1
Muhlenhoff, U.2
Lill, R.3
-
13
-
-
9644284455
-
Supramolecular assemblies of human frataxin are formed via subunit-subunit interactions mediated by a non-conserved amino-terminal region
-
O'Neill, H.A., Gakh, O. and Isaya, G. (2005) Supramolecular assemblies of human frataxin are formed via subunit-subunit interactions mediated by a non-conserved amino-terminal region. J. Mol. Biol., 345 433-439.
-
(2005)
J. Mol. Biol
, vol.345
, pp. 433-439
-
-
O'Neill, H.A.1
Gakh, O.2
Isaya, G.3
-
14
-
-
33646696625
-
Frataxin, iron-sulfur clusters, heme, ROS, and aging
-
Napoli, E., Taroni, F. and Cortopassi, G.A. (2006) Frataxin, iron-sulfur clusters, heme, ROS, and aging. Antioxid. Redox. Signal, 8, 506-516.
-
(2006)
Antioxid. Redox. Signal
, vol.8
, pp. 506-516
-
-
Napoli, E.1
Taroni, F.2
Cortopassi, G.A.3
-
15
-
-
3042763187
-
Frataxin acts as an iron chaperone protein to modulate mitochondrial aconitase activity
-
Bulteau, A.L., O'Neill, H.A., Kennedy, M.C., Ikeda-Saito, M., Isaya, G. and Szweda, L.I. (2004) Frataxin acts as an iron chaperone protein to modulate mitochondrial aconitase activity. Science, 305, 242-245.
-
(2004)
Science
, vol.305
, pp. 242-245
-
-
Bulteau, A.L.1
O'Neill, H.A.2
Kennedy, M.C.3
Ikeda-Saito, M.4
Isaya, G.5
Szweda, L.I.6
-
16
-
-
0037447390
-
Iron use for haeme synthesis is under control of the yeast frataxin homologue (Yfh1)
-
Lesuisse, E., Santos, R., Matzanke, B.F., Knight, S.A., Camadro, J.M. and Dancis, A. (2003) Iron use for haeme synthesis is under control of the yeast frataxin homologue (Yfh1). Hum. Mol. Genet., 12, 879-889.
-
(2003)
Hum. Mol. Genet
, vol.12
, pp. 879-889
-
-
Lesuisse, E.1
Santos, R.2
Matzanke, B.F.3
Knight, S.A.4
Camadro, J.M.5
Dancis, A.6
-
17
-
-
11144265730
-
Yeast frataxin solution structure, iron binding, and ferrochelatase interaction
-
He, Y., Alam, S.L., Proteasa, S.V., Zhang, Y., Lesuisse, E., Dancis, A. and Stemmler, T.L. (2004) Yeast frataxin solution structure, iron binding, and ferrochelatase interaction. Biochemistry, 43, 16254-16262.
-
(2004)
Biochemistry
, vol.43
, pp. 16254-16262
-
-
He, Y.1
Alam, S.L.2
Proteasa, S.V.3
Zhang, Y.4
Lesuisse, E.5
Dancis, A.6
Stemmler, T.L.7
-
18
-
-
2942744572
-
Frataxin-mediated iron delivery to ferrochelatase in the final step of heme biosynthesis
-
Yoon, T. and Cowan, J.A. (2004) Frataxin-mediated iron delivery to ferrochelatase in the final step of heme biosynthesis. J. Biol. Chem. 279, 25943-25946.
-
(2004)
J. Biol. Chem
, vol.279
, pp. 25943-25946
-
-
Yoon, T.1
Cowan, J.A.2
-
19
-
-
26444566405
-
Frataxin interacts functionally with mitochondrial electron transport chain proteins
-
Gonzalez-Cabo, P., Vazquez-Manrique, R.P., Garcia-Gimeno, M.A., Sanz, P. and Palau, F. (2005) Frataxin interacts functionally with mitochondrial electron transport chain proteins. Hum. Mol. Genet., 14, 2091-2098.
-
(2005)
Hum. Mol. Genet
, vol.14
, pp. 2091-2098
-
-
Gonzalez-Cabo, P.1
Vazquez-Manrique, R.P.2
Garcia-Gimeno, M.A.3
Sanz, P.4
Palau, F.5
-
20
-
-
30744458144
-
Reduction of Caenorhabditis elegans frataxin increases sensitivity to oxidative stress, reduces lifespan, and causes lethality in a mitochondrial complex II mutant
-
Vazquez-Manrique, R.P., Gonzalez-Cabo, P., Ros, S., Aziz, H., Baylis, H.A. and Palau, F. (2006) Reduction of Caenorhabditis elegans frataxin increases sensitivity to oxidative stress, reduces lifespan, and causes lethality in a mitochondrial complex II mutant. FASEB J., 20 172-174.
-
(2006)
FASEB J
, vol.20
, pp. 172-174
-
-
Vazquez-Manrique, R.P.1
Gonzalez-Cabo, P.2
Ros, S.3
Aziz, H.4
Baylis, H.A.5
Palau, F.6
-
21
-
-
0037101845
-
The yeast frataxin homolog Yfh1p plays a specific role in the maturation of cellular Fe/S protiens
-
Muhlenhoff, U., Richhardt, N., Ristow, M, Kispal, G. and Lill, R. (2002) The yeast frataxin homolog Yfh1p plays a specific role in the maturation of cellular Fe/S protiens. Hum Mol Genet, 11, 2025-2036.
-
(2002)
Hum Mol Genet
, vol.11
, pp. 2025-2036
-
-
Muhlenhoff, U.1
Richhardt, N.2
Ristow, M.3
Kispal, G.4
Lill, R.5
-
22
-
-
27944495710
-
RNAi-mediated suppression of the mitochondrial iron chaperone, frataxin, in Drosophila
-
Anderson, P.R., Kirby, K., Hilliker, A.J. and Phillips, J.P. (2005) RNAi-mediated suppression of the mitochondrial iron chaperone, frataxin, in Drosophila, Hum. Mol. Genet., 40, 3397-3405.
-
(2005)
Hum. Mol. Genet
, vol.40
, pp. 3397-3405
-
-
Anderson, P.R.1
Kirby, K.2
Hilliker, A.J.3
Phillips, J.P.4
-
23
-
-
0035138072
-
Mouse models for Friedreich ataxia exhibit cardiomyopathy, sensory nerve defect and Fe-S enzyme deficiency followed by intramitochondrial iron deposits
-
Puccio, H, Simon, D., Cossee, M, Criqui-Filipe, P., Tiziano, F., Melki, J., Hindelang, C., Matyas, R., Rustin, P. and Koenig, M. (2001) Mouse models for Friedreich ataxia exhibit cardiomyopathy, sensory nerve defect and Fe-S enzyme deficiency followed by intramitochondrial iron deposits. Nat. Genet., 27, 181-186.
-
(2001)
Nat. Genet
, vol.27
, pp. 181-186
-
-
Puccio, H.1
Simon, D.2
Cossee, M.3
Criqui-Filipe, P.4
Tiziano, F.5
Melki, J.6
Hindelang, C.7
Matyas, R.8
Rustin, P.9
Koenig, M.10
-
24
-
-
0041808717
-
-
Tan, G., Napoli, F., Taroni, F. and Cortopassi, G. (2003) Decreased expression of genes involved in sulfur amino acid metabolism in frataxin-deficient cells. Hum. Mol. Genet., 12, 1699-l711.
-
Tan, G., Napoli, F., Taroni, F. and Cortopassi, G. (2003) Decreased expression of genes involved in sulfur amino acid metabolism in frataxin-deficient cells. Hum. Mol. Genet., 12, 1699-l711.
-
-
-
-
25
-
-
30444433568
-
The Nfs1 interacting protein Isd11 has an essential role in Fe/S cluster biogenesis in mitochondria
-
Adam, A.C., Bornhovd, C., Prokisch, H., Neupert, W. and Hell, K. (2006) The Nfs1 interacting protein Isd11 has an essential role in Fe/S cluster biogenesis in mitochondria. EMBO J., 25, 174-183.
-
(2006)
EMBO J
, vol.25
, pp. 174-183
-
-
Adam, A.C.1
Bornhovd, C.2
Prokisch, H.3
Neupert, W.4
Hell, K.5
-
26
-
-
30444449009
-
Essential role of Isd11 in mitochondrial iron-sulfur cluster synthesis on Isu scaffold proteins
-
Wiedemann, N., Urzica, E., Guiard, B., Muller, H., Lohaus, C., Meyer, H.E., Ryan, M.T., Meisinger, C., Muhlenhoff, U., Lill, R. et al. (2006) Essential role of Isd11 in mitochondrial iron-sulfur cluster synthesis on Isu scaffold proteins. EMBO J., 25, 184-195.
-
(2006)
EMBO J
, vol.25
, pp. 184-195
-
-
Wiedemann, N.1
Urzica, E.2
Guiard, B.3
Muller, H.4
Lohaus, C.5
Meyer, H.E.6
Ryan, M.T.7
Meisinger, C.8
Muhlenhoff, U.9
Lill, R.10
-
27
-
-
0036241765
-
Hereditary spastic paraplegia SPG13 is associated with a mutation in the gene encoding the mitochondrial chaperonin Hsp60
-
Hansen, J.J., Durr, A., Cournu-Rebeix, I., Georgopoulos, C., Ang, D., Nielsen, M.N., Davoine, C.S., Brice, A., Fontaine, B., Gregersen, N. et al. (2002) Hereditary spastic paraplegia SPG13 is associated with a mutation in the gene encoding the mitochondrial chaperonin Hsp60. Am. J. Hum. Genet., 70, 1328-1332.
-
(2002)
Am. J. Hum. Genet
, vol.70
, pp. 1328-1332
-
-
Hansen, J.J.1
Durr, A.2
Cournu-Rebeix, I.3
Georgopoulos, C.4
Ang, D.5
Nielsen, M.N.6
Davoine, C.S.7
Brice, A.8
Fontaine, B.9
Gregersen, N.10
-
28
-
-
27444442414
-
Identification and characterization of molecular interactions between mortalin/mtHsp70 and HSP60
-
Wadhwa, R., Takano, S., Kaur, K., Aida, S., Yaguchi, T., Kaul, Z., Hirano, T., Taira, K. and Kaul, S.C. (2005) Identification and characterization of molecular interactions between mortalin/mtHsp70 and HSP60. Biochem. J., 391, 185-190.
-
(2005)
Biochem. J
, vol.391
, pp. 185-190
-
-
Wadhwa, R.1
Takano, S.2
Kaur, K.3
Aida, S.4
Yaguchi, T.5
Kaul, Z.6
Hirano, T.7
Taira, K.8
Kaul, S.C.9
-
29
-
-
0033166528
-
Identification and characterization of AFG3L2, a novel paraplegin-related gene
-
Banfi, S., Bassi, M.T., Andolfi, G., Marchitiello, A., Zanotta, S., Ballabio, A., Casari, G. and Franco, B. (1999) Identification and characterization of AFG3L2, a novel paraplegin-related gene. Genomics 59, 51-58.
-
(1999)
Genomics
, vol.59
, pp. 51-58
-
-
Banfi, S.1
Bassi, M.T.2
Andolfi, G.3
Marchitiello, A.4
Zanotta, S.5
Ballabio, A.6
Casari, G.7
Franco, B.8
-
30
-
-
0344736798
-
Loss of m-AAA protease in mitochondria causes complex I deficiency and increased sensitivity to oxidative stress in hereditary spastic paraplegia
-
Atorino, L., Silvestri, L., Koppen, M., Cassina, L., Ballabio, A., Marconi, R., Langer, T. and Casari, G. (2003) Loss of m-AAA protease in mitochondria causes complex I deficiency and increased sensitivity to oxidative stress in hereditary spastic paraplegia. J. Cell. Biol., 163, 777-787.
-
(2003)
J. Cell. Biol
, vol.163
, pp. 777-787
-
-
Atorino, L.1
Silvestri, L.2
Koppen, M.3
Cassina, L.4
Ballabio, A.5
Marconi, R.6
Langer, T.7
Casari, G.8
-
31
-
-
0042531776
-
Ssq1, a mitochondrial Hsp70 involved in iron-sulfur (Fe/S) center biogenesis. Similarities to and differences from its bacterial counterpart
-
Dutkiewicz, R., Schilke, B., Knieszner, H., Walter, W., Craig, E.A. and Marszalek, J. (2003) Ssq1, a mitochondrial Hsp70 involved in iron-sulfur (Fe/S) center biogenesis. Similarities to and differences from its bacterial counterpart. J. Biol. Chem., 278, 29719-29727.
-
(2003)
J. Biol. Chem
, vol.278
, pp. 29719-29727
-
-
Dutkiewicz, R.1
Schilke, B.2
Knieszner, H.3
Walter, W.4
Craig, E.A.5
Marszalek, J.6
-
32
-
-
26244448912
-
Extra-mitochondrial localisation of frataxin and its association with IscU1 during enterocyte-like differentiation of the human colon adenocarcinoma cell line Caco-2
-
Acquaviva, F., De Biase, I., Nezi, L., Ruggiero, G., Tatangelo, F., Pisano, C., Monticelli, A., Garbi, C., Acquaviva, A.M. and Cocozza, S. (2005) Extra-mitochondrial localisation of frataxin and its association with IscU1 during enterocyte-like differentiation of the human colon adenocarcinoma cell line Caco-2. J. Cell. Sci., 118, 3917-3924.
-
(2005)
J. Cell. Sci
, vol.118
, pp. 3917-3924
-
-
Acquaviva, F.1
De Biase, I.2
Nezi, L.3
Ruggiero, G.4
Tatangelo, F.5
Pisano, C.6
Monticelli, A.7
Garbi, C.8
Acquaviva, A.M.9
Cocozza, S.10
-
33
-
-
33745222541
-
A pool of extramitochondrial frataxin that promotes cell survival
-
Condo, I., Ventura, N., Malisan, F., Tomassini, B. and Testi, R. (2006) A pool of extramitochondrial frataxin that promotes cell survival. J. Biol. Chem., 281, 16750-16756.
-
(2006)
J. Biol. Chem
, vol.281
, pp. 16750-16756
-
-
Condo, I.1
Ventura, N.2
Malisan, F.3
Tomassini, B.4
Testi, R.5
-
34
-
-
0033838364
-
Iron-dependent self-assembly of recombinant yeast frataxin: Implications for Friedreich ataxia
-
Adamec, J., Rusnak, F., Owen, W.G., Naylor, S., Benson, L.M., Gacy, A.M. and Isaya, G. (2000) Iron-dependent self-assembly of recombinant yeast frataxin: Implications for Friedreich ataxia. Am. J. Hum. Genet., 67, 549-562.
-
(2000)
Am. J. Hum. Genet
, vol.67
, pp. 549-562
-
-
Adamec, J.1
Rusnak, F.2
Owen, W.G.3
Naylor, S.4
Benson, L.M.5
Gacy, A.M.6
Isaya, G.7
-
35
-
-
0037188390
-
Physical evidence that yeast firataxin is an iron storage protein
-
Gakh, O., Adamec, J., Gacy, A.M., Twesten, R.D., Owen, W.G. and Isaya, G. (2002) Physical evidence that yeast firataxin is an iron storage protein. Biochemistry, 41, 6798-6804.
-
(2002)
Biochemistry
, vol.41
, pp. 6798-6804
-
-
Gakh, O.1
Adamec, J.2
Gacy, A.M.3
Twesten, R.D.4
Owen, W.G.5
Isaya, G.6
-
36
-
-
0042232045
-
Yeast frataxin sequentially chaperones and stores iron by coupling protein assembly with iron oxidation
-
Park, S., Gakh, O., O'Neill, H.A., Mangravita, A., Nichol, H., Ferreira, G.C. and Isaya, G. (2003) Yeast frataxin sequentially chaperones and stores iron by coupling protein assembly with iron oxidation. J. Biol. Chem., 278, 31340-31351.
-
(2003)
J. Biol. Chem
, vol.278
, pp. 31340-31351
-
-
Park, S.1
Gakh, O.2
O'Neill, H.A.3
Mangravita, A.4
Nichol, H.5
Ferreira, G.C.6
Isaya, G.7
-
37
-
-
0037846093
-
Structure of frataxin iron cores: An X-ray absorption spectroscopic study
-
Nichol, H., Gakh, O., O'Neill, H.A., Pickering, I.J., Isaya, G. and George, G.N. (2003) Structure of frataxin iron cores: An X-ray absorption spectroscopic study. Biochemistry, 42, 5971-5976.
-
(2003)
Biochemistry
, vol.42
, pp. 5971-5976
-
-
Nichol, H.1
Gakh, O.2
O'Neill, H.A.3
Pickering, I.J.4
Isaya, G.5
George, G.N.6
-
38
-
-
33644947727
-
Colorimetric assay for the quantitation of iron in yeast
-
Tamarit, J., Irazusta, V., Moreno-Cermeno, A. and Ros, J. (2006) Colorimetric assay for the quantitation of iron in yeast. Anal. Biochem., 351, 149-151.
-
(2006)
Anal. Biochem
, vol.351
, pp. 149-151
-
-
Tamarit, J.1
Irazusta, V.2
Moreno-Cermeno, A.3
Ros, J.4
-
39
-
-
0034053969
-
Role of the mitochondrial Hsp70s, Ssc1 and Ssq1, in the maturation of Yfhl
-
Voisine, C., Schilke, B., Ohlson, M., Beinert, H., Marszalek, J. and Craig, E.A. (2000) Role of the mitochondrial Hsp70s, Ssc1 and Ssq1, in the maturation of Yfhl. Mol. Cell. Biol., 20, 3677-3684.
-
(2000)
Mol. Cell. Biol
, vol.20
, pp. 3677-3684
-
-
Voisine, C.1
Schilke, B.2
Ohlson, M.3
Beinert, H.4
Marszalek, J.5
Craig, E.A.6
-
40
-
-
0035970292
-
The mitochandrial proteins Ssq1 and Jac1 are required for the assembly of iron sulfur clusters in mitochondria
-
Lutz, T., Westermann, B., Neupert, W. and Herrmann, J.M. (2001) The mitochandrial proteins Ssq1 and Jac1 are required for the assembly of iron sulfur clusters in mitochondria. J. Mol. Biol., 307, 815-825.
-
(2001)
J. Mol. Biol
, vol.307
, pp. 815-825
-
-
Lutz, T.1
Westermann, B.2
Neupert, W.3
Herrmann, J.M.4
-
41
-
-
0032540929
-
Mt-Hsp70 homolog, Ssc2p, required for maturation of yeast frataxin and mitochondrial iron homeostasis
-
Knight, S.A., Sepuri, N.B., Pain, D. and Dancis, A. (1998) Mt-Hsp70 homolog, Ssc2p, required for maturation of yeast frataxin and mitochondrial iron homeostasis. J. Biol. Chem., 273, 18389-18393.
-
(1998)
J. Biol. Chem
, vol.273
, pp. 18389-18393
-
-
Knight, S.A.1
Sepuri, N.B.2
Pain, D.3
Dancis, A.4
-
42
-
-
3142716203
-
Sequence-specific interaction between mitochondrial Fe-S scaffold protein Isu and Hsp70 Ssq1 is essential for their in vivo function
-
Dutkiewicz, R., Schilke, B., Cheng, S., Kniezner, H., Craig, E.A. and Marszalek., J. (2004) Sequence-specific interaction between mitochondrial Fe-S scaffold protein Isu and Hsp70 Ssq1 is essential for their in vivo function. J. Biol. Chem. 279, 29167-29174.
-
(2004)
J. Biol. Chem
, vol.279
, pp. 29167-29174
-
-
Dutkiewicz, R.1
Schilke, B.2
Cheng, S.3
Kniezner, H.4
Craig, E.A.5
Marszalek, J.6
-
43
-
-
33646342998
-
The Hsp70 chaperone Ssq1p is dispensable for iron-sulfur cluster formation on the scaffold protein Isulp
-
Dutkiewicz, R., Marszalek, J., Schilke, B., Craig, E.A., Lill, R. and Muhlenhoff, U. (2006) The Hsp70 chaperone Ssq1p is dispensable for iron-sulfur cluster formation on the scaffold protein Isulp. J. Biol. Chem., 281, 7801-7808.
-
(2006)
J. Biol. Chem
, vol.281
, pp. 7801-7808
-
-
Dutkiewicz, R.1
Marszalek, J.2
Schilke, B.3
Craig, E.A.4
Lill, R.5
Muhlenhoff, U.6
-
44
-
-
0035799352
-
Combined and individual mitochondrial HSP60 and HSP10 expression in cardiac myocytes protects mitochondrial function and prevents apoptotic cell deaths induced by simulated ischemia-reoxygenation
-
Lin, K.M., Lin, B., Lian, I.Y., Mestril, R., Scheffler, I.E. and Dillmann W.H. (2001) Combined and individual mitochondrial HSP60 and HSP10 expression in cardiac myocytes protects mitochondrial function and prevents apoptotic cell deaths induced by simulated ischemia-reoxygenation. Circulation, 103, 1787-1792.
-
(2001)
Circulation
, vol.103
, pp. 1787-1792
-
-
Lin, K.M.1
Lin, B.2
Lian, I.Y.3
Mestril, R.4
Scheffler, I.E.5
Dillmann, W.H.6
-
45
-
-
0032924105
-
Chaperone-mediated protein folding
-
Fink A.L. (1999) Chaperone-mediated protein folding. Physiol. Rev. 79, 425-449.
-
(1999)
Physiol. Rev
, vol.79
, pp. 425-449
-
-
Fink, A.L.1
-
46
-
-
0037449707
-
Active rhodanese lacking nonessential sulfhydryl groups contains an unstable C-terminal domain and can be bound, inactivated, and reactivated by GroEL
-
Ybarra, J., Bhattacharyya, A.M., Panda, M. and Horowitz, P.M. (2003) Active rhodanese lacking nonessential sulfhydryl groups contains an unstable C-terminal domain and can be bound, inactivated, and reactivated by GroEL. J. Biol. Chem., 278, 1693-1699.
-
(2003)
J. Biol. Chem
, vol.278
, pp. 1693-1699
-
-
Ybarra, J.1
Bhattacharyya, A.M.2
Panda, M.3
Horowitz, P.M.4
-
47
-
-
0021799917
-
Enzymic synthesis of the 4Fe-4S clusters of Clostridium pasteurianum ferredoxin
-
Bonomi, F., Pagani, S., and Kurtz, D.M., Jr. (1985) Enzymic synthesis of the 4Fe-4S clusters of Clostridium pasteurianum ferredoxin. Eur. J. Biochem., 148, 67-73.
-
(1985)
Eur. J. Biochem
, vol.148
, pp. 67-73
-
-
Bonomi, F.1
Pagani, S.2
Kurtz Jr., D.M.3
-
48
-
-
0035011524
-
GroEL-assisted refolding of adrenodoxin during chemical cluster insertion
-
Iametti, S., Bera, A.K., Vecchio, G., Grinberg, A., Bernhardt, R. and Bonomi, F. (2001) GroEL-assisted refolding of adrenodoxin during chemical cluster insertion. Eur. J. Biochem., 268, 2421-2429.
-
(2001)
Eur. J. Biochem
, vol.268
, pp. 2421-2429
-
-
Iametti, S.1
Bera, A.K.2
Vecchio, G.3
Grinberg, A.4
Bernhardt, R.5
Bonomi, F.6
-
49
-
-
0037113995
-
Mitochondrial Hsp60, resistance to oxidative stress, and the labile iron pool are closely connected in Saccharomyces cerevisiae
-
Cabiscol, E., Belli, G., Tamarit, J., Echave, P., Herrero, E. and Ros, J. (2002) Mitochondrial Hsp60, resistance to oxidative stress, and the labile iron pool are closely connected in Saccharomyces cerevisiae. J. Biol. Chem., 277, 44531-44538.
-
(2002)
J. Biol. Chem
, vol.277
, pp. 44531-44538
-
-
Cabiscol, E.1
Belli, G.2
Tamarit, J.3
Echave, P.4
Herrero, E.5
Ros, J.6
-
50
-
-
33747170368
-
Evolution of mitochondrial chaperones utilized in Fe-S chister biogenesis
-
Schilke, B., Williams, B., Knieszner, H., Pukszta, S., D'Silva, P., Craig, E.A. and Marszalek, J. (2006) Evolution of mitochondrial chaperones utilized in Fe-S chister biogenesis. Curr. Biol., 16 1660-1665.
-
(2006)
Curr. Biol
, vol.16
, pp. 1660-1665
-
-
Schilke, B.1
Williams, B.2
Knieszner, H.3
Pukszta, S.4
D'Silva, P.5
Craig, E.A.6
Marszalek, J.7
-
51
-
-
0021669709
-
-
Anke, M., Groppel, B., Kronemann, H. and Grün, M. (1984) Nickel - an essential element. In Sunderman, F.W., Jr. (Ed.), Nickel in the Human Environment. IARC Sci. Publ. 53, Lyon, pp. 339-365.
-
Anke, M., Groppel, B., Kronemann, H. and Grün, M. (1984) Nickel - an essential element. In Sunderman, F.W., Jr. (Ed.), Nickel in the Human Environment. IARC Sci. Publ. 53, Lyon, pp. 339-365.
-
-
-
-
52
-
-
22544480812
-
Nickel decreases cellular iron level and converts cytosolic aconitase to iron-regulatory protein 1 in A549 cells
-
Chen, H., Davidson, T., Singleton, S., Garrick, M.D. and Costa, M. (2005) Nickel decreases cellular iron level and converts cytosolic aconitase to iron-regulatory protein 1 in A549 cells. Toxicol. Appl. Pharmacol., 206, 275-287.
-
(2005)
Toxicol. Appl. Pharmacol
, vol.206
, pp. 275-287
-
-
Chen, H.1
Davidson, T.2
Singleton, S.3
Garrick, M.D.4
Costa, M.5
-
53
-
-
0038203196
-
Nickel uptake and utilization by microorganisms
-
Mulrooney, S.B. and Hausinger, R.P. (2003) Nickel uptake and utilization by microorganisms. FEMS Microbiol. Rev., 27, 239-261.
-
(2003)
FEMS Microbiol. Rev
, vol.27
, pp. 239-261
-
-
Mulrooney, S.B.1
Hausinger, R.P.2
-
54
-
-
0036941310
-
Stability and nickel binding properties of peptides designed as scaffolds for the stabilization of Ni(II)-Fe(4)S(4) bridged assemblies
-
Laplaza, C.E. and Holm, R.H. (2002) Stability and nickel binding properties of peptides designed as scaffolds for the stabilization of Ni(II)-Fe(4)S(4) bridged assemblies. J. Biol. Inorg. Chem., 7 451-460.
-
(2002)
J. Biol. Inorg. Chem
, vol.7
, pp. 451-460
-
-
Laplaza, C.E.1
Holm, R.H.2
-
55
-
-
0344820730
-
Friedreich's ataxia: Point mutations and clinical presentation of compound heterozygotes
-
Cossee, M., Durr, A., Schmitt, M., Dahl, N., Trouillas, P., Allinson, P., Koshzewa, M., Nivelon-Chevallier, A., Gustavson, K.H., Kohlschutter, A. et al. (1999) Friedreich's ataxia: Point mutations and clinical presentation of compound heterozygotes. Ann. Neurol., 45, 200-206.
-
(1999)
Ann. Neurol
, vol.45
, pp. 200-206
-
-
Cossee, M.1
Durr, A.2
Schmitt, M.3
Dahl, N.4
Trouillas, P.5
Allinson, P.6
Koshzewa, M.7
Nivelon-Chevallier, A.8
Gustavson, K.H.9
Kohlschutter, A.10
-
56
-
-
0034613233
-
Crystal structure of human frataxin
-
Dhe-Paganon, S., Shigeta, R., Chi, Y.I., Ristow, M. and Shoelson, S.E. (2000) Crystal structure of human frataxin. J. Biol. Chem., 275 30753-30756.
-
(2000)
J. Biol. Chem
, vol.275
, pp. 30753-30756
-
-
Dhe-Paganon, S.1
Shigeta, R.2
Chi, Y.I.3
Ristow, M.4
Shoelson, S.E.5
-
57
-
-
7944225865
-
Solution structure of the bacterial frataxin ortholog, CyaY: Mapping the iron binding sites
-
Nair, M., Adinolfi S., Pastore, C., Kelly, G., Temussi, P. and Pastore, A. (2004) Solution structure of the bacterial frataxin ortholog, CyaY: mapping the iron binding sites. Structure, 12, 2037-2048.
-
(2004)
Structure
, vol.12
, pp. 2037-2048
-
-
Nair, M.1
Adinolfi, S.2
Pastore, C.3
Kelly, G.4
Temussi, P.5
Pastore, A.6
-
58
-
-
0005622907
-
A missense mutation (W155R) in an American patient with Friedreich Ataxia
-
Labuda, M., Poirier, J. and Pandolfo, M. (1999) A missense mutation (W155R) in an American patient with Friedreich Ataxia. Hum. mutat. 13, 506-506.
-
(1999)
Hum. mutat
, vol.13
, pp. 506-506
-
-
Labuda, M.1
Poirier, J.2
Pandolfo, M.3
-
59
-
-
33745217828
-
iron-sulfur cluster biosynthesis: Characterization of Escherichia coli CYaY as an iron donor for the assembly of [2Fe-2S] clusters in the scaffold IscU
-
Layer, G., Ollagnier-de Choudens, S., Sanakis, Y. and Fontecave, M. (2006) iron-sulfur cluster biosynthesis: Characterization of Escherichia coli CYaY as an iron donor for the assembly of [2Fe-2S] clusters in the scaffold IscU. J. Biol. Chem., 281, 16256-16263.
-
(2006)
J. Biol. Chem
, vol.281
, pp. 16256-16263
-
-
Layer, G.1
Ollagnier-de Choudens, S.2
Sanakis, Y.3
Fontecave, M.4
-
60
-
-
33745618402
-
Evolution of the Isd11-IscS complex reveals a single alpha-proteobacterial endosymbiosis for all eukaryotes
-
Richards, T.A. and van der Giezen, M. (2006) Evolution of the Isd11-IscS complex reveals a single alpha-proteobacterial endosymbiosis for all eukaryotes. Mol. Biol. Evol., 23, 1341-1344.
-
(2006)
Mol. Biol. Evol
, vol.23
, pp. 1341-1344
-
-
Richards, T.A.1
van der Giezen, M.2
-
61
-
-
29644442275
-
Frataxin deficiency alters heme pathway trancripts and decreases mitochondrial heme metabolites in mammalian cells
-
Schoenfeld, R.A., Napoli, E., Wong, A., Zhan, S., Reutenauer, L., Morin, D., Buckpitt, A.R., Taroni, F., Lonnerdal, B., Ristow, M. et al. (2005) Frataxin deficiency alters heme pathway trancripts and decreases mitochondrial heme metabolites in mammalian cells. Hum. Mol. Genet. 14, 3787-3799.
-
(2005)
Hum. Mol. Genet
, vol.14
, pp. 3787-3799
-
-
Schoenfeld, R.A.1
Napoli, E.2
Wong, A.3
Zhan, S.4
Reutenauer, L.5
Morin, D.6
Buckpitt, A.R.7
Taroni, F.8
Lonnerdal, B.9
Ristow, M.10
-
62
-
-
0034778511
-
Frataxin expression rescues mitochondrial dysfunctions in FRDA cells
-
Tan, G., Chen, L.S., Lonnerdal, B., Gellera, C., Taroni, F.A. and Cortopassi, G.A. (2001) Frataxin expression rescues mitochondrial dysfunctions in FRDA cells. Hum. Mol. Genet., 10, 2099-2107.
-
(2001)
Hum. Mol. Genet
, vol.10
, pp. 2099-2107
-
-
Tan, G.1
Chen, L.S.2
Lonnerdal, B.3
Gellera, C.4
Taroni, F.A.5
Cortopassi, G.A.6
-
63
-
-
0036361362
-
Reproducible quantitative PCR of mitochondrial and nuclear DNA copy number using the LightCycler
-
Wong, A. and Cortopassi, G. (2002) Reproducible quantitative PCR of mitochondrial and nuclear DNA copy number using the LightCycler. Methods Mol. Biol., 197, 129-137.
-
(2002)
Methods Mol. Biol
, vol.197
, pp. 129-137
-
-
Wong, A.1
Cortopassi, G.2
-
64
-
-
0036351317
-
Analysis of gene function in somatic mammalian cells using small interfering RNAs
-
Elbashir, S.M., Harborth, J., Weber, K. and Tuschl, T. (2002) Analysis of gene function in somatic mammalian cells using small interfering RNAs. Methods, 26, 199-213.
-
(2002)
Methods
, vol.26
, pp. 199-213
-
-
Elbashir, S.M.1
Harborth, J.2
Weber, K.3
Tuschl, T.4
-
65
-
-
0023899855
-
Rapid colorimetric micromethod for the quantitation of complexed iron in biological samples
-
Fish, W.W. (1988) Rapid colorimetric micromethod for the quantitation of complexed iron in biological samples. Methods Enzymol., 158, 357-364.
-
(1988)
Methods Enzymol
, vol.158
, pp. 357-364
-
-
Fish, W.W.1
-
66
-
-
33644623262
-
Functions of mitochondrial ISCU and cytosolic ISCU in mammalian iron-sulfur cluster biogenesis and iron homeostasis
-
Tong, W.H. and Rousult, T.A. (2006) Functions of mitochondrial ISCU and cytosolic ISCU in mammalian iron-sulfur cluster biogenesis and iron homeostasis. Cell. Metab., 3, 199-210.
-
(2006)
Cell. Metab
, vol.3
, pp. 199-210
-
-
Tong, W.H.1
Rousult, T.A.2
|