-
1
-
-
84878921567
-
Mutation of the iron-sulfur cluster assembly gene iba57 causes severe myopathy and encephalopathy
-
Ajit Bolar N, Vanlander AV, Wilbrecht C, et al. (2013). Mutation of the iron-sulfur cluster assembly gene IBA57 causes severe myopathy and encephalopathy. Hum Mol Genet 22: 2590-602
-
(2013)
Hum Mol Genet
, vol.22
, pp. 2590-2602
-
-
Ajit Bolar, N.1
Vanlander, A.V.2
Wilbrecht, C.3
-
2
-
-
84870281311
-
Recessive germline sdha and sdhb mutations causing leukodystrophy and isolated mitochondrial complex ii deficiency
-
Alston CL, Davisonje, Meloni F, et al. (2012). Recessive germline SDHA and SDHB mutations causing leukodystrophy and isolated mitochondrial complex II deficiency. J Med Gen 49: 569-77
-
(2012)
J Med Gen
, vol.49
, pp. 569-577
-
-
Alston, C.L.1
Davisonj, E.2
Meloni, F.3
-
3
-
-
0034964421
-
Gene mutations in the succinate dehydrogenase subunit sdhb cause susceptibility to familial pheochromocytoma and to familial paraganglioma
-
Astuti D, Latif F, Dallol A, et al. (2001). Gene mutations in the succinate dehydrogenase subunit SDHB cause susceptibility to familial pheochromocytoma and to familial paraganglioma. Am J Hum Genet 69: 49-54
-
(2001)
Am J Hum Genet
, vol.69
, pp. 49-54
-
-
Astuti, D.1
Latif, F.2
Dallol, A.3
-
4
-
-
77953531870
-
Mzm1 influences a labile pool of mitochondrial zinc important for respiratory function
-
Atkinson A, Khalimonchuk O, Smith P, et al. (2010). Mzm1 influences a labile pool of mitochondrial zinc important for respiratory function. J Biol Chem 285: 19450-9
-
(2010)
J Biol Chem
, vol.285
, pp. 19450-19459
-
-
Atkinson, A.1
Khalimonchuk, O.2
Smith, P.3
-
5
-
-
0347683482
-
Riboflavin uptake and fad synthesis in saccharomyces cerevisiae mitochondria: Involvement of the flx1p carrier in fad export
-
Bafunno V, Giancaspero TA, Brizio C, et al. (2004). Riboflavin uptake and FAD synthesis in Saccharomyces cerevisiae mitochondria: involvement of the Flx1p carrier in FAD export. J Biol Chem 279: 95-102
-
(2004)
J Biol Chem
, vol.279
, pp. 95-102
-
-
Bafunno, V.1
Giancaspero, T.A.2
Brizio, C.3
-
6
-
-
84893819382
-
Variant non ketotic hyperglycinemia is caused by mutations in lias, bola3 and the novel gene glrx5
-
Baker II PR,, Friederich MW, Swanson MA, et al. (2014). Variant non ketotic hyperglycinemia is caused by mutations in LIAS, BOLA3 and the novel gene GLRX5. Brain 137: 366-79
-
(2014)
Brain
, vol.137
, pp. 366-379
-
-
Baker, I.I.P.R.1
Friederich, M.W.2
Swanson, M.A.3
-
8
-
-
0034602950
-
Mutations in sdhd, a mitochondrial complex ii gene, in hereditary paraganglioma
-
Baysal BE. (2000). Mutations in SDHD, a mitochondrial complex II gene, in hereditary paraganglioma. Science 287: 848-51
-
(2000)
Science
, vol.287
, pp. 848-851
-
-
Baysal, B.E.1
-
9
-
-
0029159804
-
Mutation of a nuclear succinate dehydrogenase gene results in mitochondrial respiratory chain deficiency
-
Bourgeron T, Rustin P, Chretien D, et al. (1995). Mutation of a nuclear succinate dehydrogenase gene results in mitochondrial respiratory chain deficiency. Nat Genet 11: 144-9
-
(1995)
Nat Genet
, vol.11
, pp. 144-149
-
-
Bourgeron, T.1
Rustin, P.2
Chretien, D.3
-
10
-
-
0024347725
-
Covalent cofactor binding to flavoenzymes requires specific effectors
-
Brandsch R, Bichler V. (1989). Covalent cofactor binding to flavoenzymes requires specific effectors. Eur J Biochem 182: 125-8
-
(1989)
Eur J Biochem
, vol.182
, pp. 125-128
-
-
Brandsch, R.1
Bichler, V.2
-
11
-
-
77958164441
-
Sdha is a tumor suppressor gene causing paraganglioma
-
Burnichon N, Briere JJ, Libe R, et al. (2010). SDHA is a tumor suppressor gene causing paraganglioma. Hum Mol Genet 19: 3011-20
-
(2010)
Hum Mol Genet
, vol.19
, pp. 3011-3020
-
-
Burnichon, N.1
Briere, J.J.2
Libe, R.3
-
12
-
-
80053898097
-
Mutations in ironsulfur cluster scaffold genes nfu1 and bola3 cause a fatal deficiency of multiple respiratory chain and 2-oxoacid dehydrogenase enzymes
-
Cameron JM, Janer A, Levandovskiy V, et al. (2011). Mutations in ironsulfur cluster scaffold genes NFU1 and BOLA3 cause a fatal deficiency of multiple respiratory chain and 2-oxoacid dehydrogenase enzymes. Am J Hum Genet 89: 486-95
-
(2011)
Am J Hum Genet
, vol.89
, pp. 486-495
-
-
Cameron, J.M.1
Janer, A.2
Levandovskiy, V.3
-
14
-
-
84857627270
-
Interaction of j-protein co-chaperone jac1 with fe-s scaffold isu is indispensable in vivo and conserved in evolution
-
Ciesielski SJ, Schilke BA, Osipiuk J, et al. (2012). Interaction of J-protein co-chaperone Jac1 with Fe-S scaffold Isu is indispensable in vivo and conserved in evolution. J Mol Biol 417: 1-12
-
(2012)
J Mol Biol
, vol.417
, pp. 1-12
-
-
Ciesielski, S.J.1
Schilke, B.A.2
Osipiuk, J.3
-
15
-
-
84870040471
-
Tissue specificity of a human mitochondrial disease: Differentiation-enhanced mis-splicing of the fe-s scaffold gene iscu renders patient cells more sensitive to oxidative stress in iscu myopathy
-
Crooks DR, Jeong SY, Tong WH, et al. (2012). Tissue specificity of a human mitochondrial disease: differentiation-enhanced mis-splicing of the Fe-S scaffold gene ISCU renders patient cells more sensitive to oxidative stress in ISCU myopathy. J Biol Chem 287: 40119-30
-
(2012)
J Biol Chem
, vol.287
, pp. 40119-40130
-
-
Crooks, D.R.1
Jeong, S.Y.2
Tong, W.H.3
-
16
-
-
79959861353
-
Mitochondrial disorders caused by mutations in respiratory chain assembly factors
-
Diaz F, Kotarsky H, Fellman V, Moraes CT. (2011). Mitochondrial disorders caused by mutations in respiratory chain assembly factors. Semin Fetal Neonatal Med 16: 197-204
-
(2011)
Semin Fetal Neonatal Med
, vol.16
, pp. 197-204
-
-
Diaz, F.1
Kotarsky, H.2
Fellman, V.3
Moraes, C.T.4
-
17
-
-
84871792997
-
Mitochondrial protein import: Common principles and physiological networks
-
Dudek J, Rehling P, van der Laan M. (2013). Mitochondrial protein import: common principles and physiological networks. BBA-Mol Cell Res 1833: 274-85
-
(2013)
BBA-Mol Cell Res
, vol.1833
, pp. 274-285
-
-
Dudek, J.1
Rehling, P.2
Van Der Laan, M.3
-
18
-
-
84868120924
-
Solution nmr structure of yeast succinate dehydrogenase flavinylation factor sdh5 reveals a putative sdh1 binding site
-
eletsky A, Jeong M-Y, Kim H, et al. (2012). Solution NMR structure of yeast succinate dehydrogenase flavinylation factor Sdh5 reveals a putative Sdh1 binding site. Biochemistry 51: 8475-7
-
(2012)
Biochemistry
, vol.51
, pp. 8475-8477
-
-
Eletsky, A.1
Jeong, M.-Y.2
Kim, H.3
-
19
-
-
56349124605
-
Assembly of the oxidative phosphorylation system in humans: What we have learned by studying its defects
-
Fernandez-Vizarra E, Tiranti V, Zeviani M. (2009). Assembly of the oxidative phosphorylation system in humans: what we have learned by studying its defects. Biochim Biophys Acta 1793: 200-11
-
(2009)
Biochim Biophys Acta
, vol.1793
, pp. 200-211
-
-
Fernandez-Vizarra, E.1
Tiranti, V.2
Zeviani, M.3
-
20
-
-
84884355744
-
Protein expression profiles in patients carrying NFU1 mutation. Contribution to the pathophysiology of the disease
-
Ferrer-Cortes X, Font A, Bujan N, et al. (2013). Protein expression profiles in patients carrying NFU1 mutations. Contribution to the pathophysiology of the disease. J Inherit Metab Dis 36: 841-7
-
(2013)
J Inherit Metab Dis
, vol.36
, pp. 841-847
-
-
Ferrer-Cortes, X.1
Font, A.2
Bujan, N.3
-
21
-
-
40749086415
-
Mitochondrial iba57p is required for fe/s cluster formation on aconitase and activation of radical sam enzymes
-
Gelling C, Dawes IW, Richhardt N, et al. (2008). Mitochondrial Iba57p is required for Fe/S cluster formation on aconitase and activation of radical SAM enzymes. Mol Cell Biol 28: 1851-61
-
(2008)
Mol Cell Biol
, vol.28
, pp. 1851-1861
-
-
Gelling, C.1
Dawes, I.W.2
Richhardt, N.3
-
22
-
-
67349189168
-
Sdhaf1, encoding a lyr complex-ii specific assembly factor, is mutated in sdh-defective infantile leukoencephalopathy
-
Ghezzi D, Goffrini P, Uziel G, et al. (2009). SDHAF1, encoding a LYR complex-II specific assembly factor, is mutated in SDH-defective infantile leukoencephalopathy. Nat Genet 41: 654-6
-
(2009)
Nat Genet
, vol.41
, pp. 654-656
-
-
Ghezzi, D.1
Goffrini, P.2
Uziel, G.3
-
23
-
-
37849022071
-
Loss of the sdhb, but not the sdha, subunit of complex ii triggers reactive oxygen speciesdependenthypoxia-inducible factor activation and tumorigenesis
-
Guzy RD, SharmAb, Bell E, et al. (2008). Loss of the SdhB, but Not the SdhA, subunit of complex II triggers reactive oxygen speciesdependenThypoxia-inducible factor activation and tumorigenesis. Mol Cell Biol 28: 718-31
-
(2008)
Mol Cell Biol
, vol.28
, pp. 718-731
-
-
Guzy, R.D.1
Sharm, A.B.2
Bell, E.3
-
24
-
-
0343052744
-
Succinate: Quinone oxidoreductases. Variations on a censerved theme
-
hA& D; Die;gerhA& D; Die;ll C. (1997). Succinate: quinone oxidoreductases. variations on a censerved theme. Biochim Biophys Acta 1320: 107-41
-
(1997)
Biochim Biophys Acta
, vol.1320
, pp. 107-141
-
-
Ha, D.1
Die Gerha, D.2
Diell, C.3
-
25
-
-
0027145130
-
Mitochondrial myopathy with succinate dehydrogenase and aconitase deficiency abnormalities of several iron-sulfur proteins
-
hall RE, Henriksson KG, Lewis SF, et al. (1993). Mitochondrial myopathy with succinate dehydrogenase and aconitase deficiency. Abnormalities of several iron-sulfur proteins. J Clin Invest 92: 2660-6
-
(1993)
J Clin Invest
, vol.92
, pp. 2660-2666
-
-
Hall, R.E.1
Henriksson, K.G.2
Lewis, S.F.3
-
26
-
-
69549088424
-
Sdh5, a gene required for flavination of succinate dehydrogenase, is mutated in paraganglioma
-
hao HX, Khalimonchuk O, Schraders M, et al. (2009). SDH5, a gene required for flavination of succinate dehydrogenase, is mutated in paraganglioma. Science 325: 1139-42
-
(2009)
Science
, vol.325
, pp. 1139-1142
-
-
Hao, H.X.1
Khalimonchuk, O.2
Schraders, M.3
-
28
-
-
29644447089
-
Leigh syndrome caused by mutations in the flavoprotein (fp) subunit of succinate dehydrogenase (sdha
-
horváth R, Abicht A, Holinski-Feder E, et al. (2006). Leigh syndrome caused by mutations in the flavoprotein (Fp) subunit of succinate dehydrogenase (SDHA). J Neurol Neurosurg Psychiatry 77: 74-6
-
(2006)
J Neurol Neurosurg Psychiatry
, vol.77
, pp. 74-76
-
-
Horváth, R.1
Abicht, A.2
Holinski-Feder, E.3
-
29
-
-
33645065589
-
Iron-sulphur clusters and the problem with oxygen
-
Imlay JA. (2006). Iron-sulphur clusters and the problem with oxygen. Mol Microbiol 59: 1073-82
-
(2006)
Mol Microbiol
, vol.59
, pp. 1073-1082
-
-
Imlay, J.A.1
-
30
-
-
11244279161
-
A mutation in the sdhc gene of complex ii increases oxidative stress, resulting in apoptosis and tumorigenesis
-
Ishii T, Yasuda K, Akatsuka A, et al. (2005). A mutation in the SDHC gene of complex II increases oxidative stress, resulting in apoptosis and tumorigenesis. Cancer Res 65: 203-9
-
(2005)
Cancer Res
, vol.65
, pp. 203-209
-
-
Ishii, T.1
Yasuda, K.2
Akatsuka, A.3
-
31
-
-
84894412950
-
Mutations in sdhd lead to autosomal recessive encephalomyopathy and isolated mitochondrial complex ii deficiency
-
Jackson CB, Nuoffer J-M, Hahn D, et al. (2014). Mutations in SDHD lead to autosomal recessive encephalomyopathy and isolated mitochondrial complex II deficiency. J Med Genet 51: 170-5
-
(2014)
J Med Genet
, vol.51
, pp. 170-175
-
-
Jackson, C.B.1
Nuoffer, J.-M.2
Hahn, D.3
-
32
-
-
84872947433
-
Complex ii deficiency-A case report and review of the literature
-
Jain-Ghai S, Cameron JM, Al Maawali A, et al. (2013). Complex II deficiency-A case report and review of the literature. Am J Med Genet A 161A: 285-94
-
(2013)
Am J Med Genet A
, vol.161 A
, pp. 285-294
-
-
Jain-Ghai, S.1
Cameron, J.M.2
Al Maawali, A.3
-
33
-
-
78651082042
-
Defects in succinate dehydrogenase in gastrointestinal stromal tumors lacking kit and pdgfra mutations
-
Janeway KA, Kim SY, Lodish M, et al. (2011). Defects in succinate dehydrogenase in gastrointestinal stromal tumors lacking KIT and PDGFRA mutations. Proc Natl Acad Sci USA 108: 314-18
-
(2011)
Proc Natl Acad Sci USA
, vol.108
, pp. 314-318
-
-
Janeway, K.A.1
Kim, S.Y.2
Lodish, M.3
-
34
-
-
0034107324
-
Role of saccharomyces cerevisiae isa1and isa2 in iron homeostasis
-
Jensen LT, Culotta VC. (2000). Role of Saccharomyces cerevisiae ISA1and ISA2 in iron homeostasis. Mol Cell Biol 20: 3918-27
-
(2000)
Mol Cell Biol
, vol.20
, pp. 3918-3927
-
-
Jensen, L.T.1
Culotta, V.C.2
-
35
-
-
78651265091
-
The crystal structure of human glrx5: Iron-sulfur cluster co-ordination, tetrameric assembly and monomer activity
-
Johansson C, Roos AK, Montano SJ, et al. (2011). The crystal structure of human GLRX5: iron-sulfur cluster co-ordination, tetrameric assembly and monomer activity. Biochem J 433: 303-11
-
(2011)
Biochem J
, vol.433
, pp. 303-311
-
-
Johansson, C.1
Roos, A.K.2
Montano, S.J.3
-
36
-
-
0021112587
-
Beinerth The role of iron in the activation-inactivation of aconitase
-
Kennedy MC, Emptage MH, Dreyer JL, BeinerTh. (1983). The role of iron in the activation-inactivation of aconitase. J Biol Chem 258: 11098-105
-
(1983)
J Biol Chem
, vol.258
, pp. 11098-11105
-
-
Kennedy, M.C.1
Emptage, M.H.2
Dreyer, J.L.3
-
37
-
-
84870012890
-
Flavinylation and assembly of succinate dehydrogenase are dependent on the c-Terminal tail of the flavoprotein subunit
-
Kim HJ, Jeong MY, Na U, Winge DR. (2012). Flavinylation and assembly of succinate dehydrogenase are dependent on the C-Terminal tail of the flavoprotein subunit. J Biol Chem 287: 40670-9
-
(2012)
J Biol Chem
, vol.287
, pp. 40670-40679
-
-
Kim, H.J.1
Jeong, M.Y.2
Na, U.3
Winge, D.R.4
-
38
-
-
84875737632
-
Emerging concepts in the flavinylation of succinate dehydrogenase
-
Kim HJ, Winge DR. (2013). Emerging concepts in the flavinylation of succinate dehydrogenase. Biochim Biophys Acta 1827: 627-36
-
(2013)
Biochim Biophys Acta
, vol.1827
, pp. 627-636
-
-
Kim, H.J.1
Winge, D.R.2
-
39
-
-
84896090117
-
Analysis of covalent flavinylation using thermostable succinate dehydrogenase from thermus thermophilus and sulfolobus tokodaii lacking sdhe homologs
-
Kounosu A. (2014). Analysis of covalent flavinylation using thermostable succinate dehydrogenase from Thermus thermophilus and Sulfolobus tokodaii lacking SdhE homologs. FEBS Lett 588: 1058-63
-
(2014)
FEBS Lett
, vol.588
, pp. 1058-1063
-
-
Kounosu, A.1
-
40
-
-
78651282654
-
Specific disintegration of complex ii succinate: Ubiquinone oxidoreductase links ph changes to oxidative stress for apoptosis induction
-
Lemarie A, Huc L, Pazarentzos E, et al. (2011). Specific disintegration of complex II succinate: ubiquinone oxidoreductase links pH changes to oxidative stress for apoptosis induction. Cell Death Differ 18: 338-49
-
(2011)
Cell Death Differ
, vol.18
, pp. 338-349
-
-
Lemarie, A.1
Huc, L.2
Pazarentzos, E.3
-
41
-
-
0037122946
-
The saccharomyces cerevisiae mitochondrial succinate: Ubiquinone oxidoreductase
-
Lemire BD, Oyedotun KS. (2002). The Saccharomyces cerevisiae mitochondrial succinate: ubiquinone oxidoreductase. Biochim Biophys Acta 1553: 102-16
-
(2002)
Biochim Biophys Acta
, vol.1553
, pp. 102-116
-
-
Lemire, B.D.1
Oyedotun, K.S.2
-
42
-
-
84861850380
-
Monothiol cgfs glutaredoxins and bola-like proteins: [2fe-2s] binding partners in iron homeostasis
-
Li H, Outten CE. (2012). Monothiol CGFS glutaredoxins and BolA-like proteins: [2Fe-2S] binding partners in iron homeostasis. Biochemistry 51: 4377-89
-
(2012)
Biochemistry
, vol.51
, pp. 4377-4389
-
-
Li, H.1
Outten, C.E.2
-
43
-
-
84864296714
-
The role of mitochondria in cellular iron-sulfur protein biogenesis and iron metabolism
-
Lill R, Hoffmann B, Molik S, et al. (2012). The role of mitochondria in cellular iron-sulfur protein biogenesis and iron metabolism. Biochim Biophys Acta 1823: 1491-508
-
(2012)
Biochim Biophys Acta
, vol.1823
, pp. 1491-1508
-
-
Lill, R.1
Hoffmann, B.2
Molik, S.3
-
44
-
-
84886998416
-
Mutations in lyrm4, encoding iron-sulfur cluster biogenesis factor isd11, cause deficiency of multiple respiratory chain complexes
-
Lim SC, Friemel M, Marum JE, et al. (2013). Mutations in LYRM4, encoding iron-sulfur cluster biogenesis factor ISD11, cause deficiency of multiple respiratory chain complexes. Hum Mol Genet 22: 4460-73
-
(2013)
Hum Mol Genet
, vol.22
, pp. 4460-4473
-
-
Lim, S.C.1
Friemel, M.2
Marum, J.E.3
-
45
-
-
84895735383
-
Cochaperone binding to lyr motifs confers specificity of iron sulfur cluster delivery
-
Maio N, Singh A, UhrigshardTh, et al. (2014). Cochaperone binding to LYR motifs confers specificity of iron sulfur cluster delivery. Cell Metab 19: 445-57
-
(2014)
Cell Metab
, vol.19
, pp. 445-457
-
-
Maio, N.1
Singh, A.2
Uhrigshard, T.H.3
-
46
-
-
84885125562
-
Binding of the chaperone jac1 protein and cysteine desulfurase nfs1 to the ironsulfur cluster scaffold isu protein is mutually exclusive
-
Majewska J, Ciesielski SJ, Schilke B, et al. (2013). Binding of the chaperone Jac1 protein and cysteine desulfurase Nfs1 to the ironsulfur cluster scaffold Isu protein is mutually exclusive. J Biol Chem 288: 29134-42
-
(2013)
J Biol Chem
, vol.288
, pp. 29134-29142
-
-
Majewska, J.1
Ciesielski, S.J.2
Schilke, B.3
-
47
-
-
0031009910
-
Shy1, the yeasthomolog of the mammalian surf-1 gene, encodes a mitochondrial protein required for respiration
-
Mashkevich G, Repetto B, Glerum DM, et al. (1997). SHY1, the yeasThomolog of the mammalian SURF-1 gene, encodes a mitochondrial protein required for respiration. J Biol Chem 272: 14356-64
-
(1997)
J Biol Chem
, vol.272
, pp. 14356-14364
-
-
Mashkevich, G.1
Repetto, B.2
Glerum, D.M.3
-
48
-
-
0037044847
-
Mechanism of superoxide and hydrogen peroxide formation by fumarate reductase, succinate dehydrogenase, and aspartate oxidase
-
Messner KR, Imlay JA. (2002). Mechanism of superoxide and hydrogen peroxide formation by fumarate reductase, succinate dehydrogenase, and aspartate oxidase. J Biol Chem 277: 42563-71
-
(2002)
J Biol Chem
, vol.277
, pp. 42563-42571
-
-
Messner, K.R.1
Imlay, J.A.2
-
49
-
-
80955125480
-
Specialized function of yeast isa1 and isa2 proteins in the maturation of mitochondrial [4fe-4s] proteins
-
Muhlenhoff U, Richter N, Pines O, et al. (2011). Specialized function of yeast Isa1 and Isa2 proteins in the maturation of mitochondrial [4Fe-4S] proteins. J Biol Chem 286: 41205-16
-
(2011)
J Biol Chem
, vol.286
, pp. 41205-41216
-
-
Muhlenhoff, U.1
Richter, N.2
Pines, O.3
-
50
-
-
84905860097
-
The lyr factors sdhaf1 and sdhaf3 mediate maturation of the iron-sulfur subunit of succinate dehydrogenase
-
Na U, Yu W, Cox JE, et al. (2014). The LYR factors SDHAF1 and SDHAF3 mediate maturation of the iron-sulfur subunit of succinate dehydrogenase. Cell Metab 20: 253-66
-
(2014)
Cell Metab
, vol.20
, pp. 253-266
-
-
Na, U.1
Yu, W.2
Cox, J.E.3
-
51
-
-
80955133245
-
A fatal mitochondrial disease is associated with defective nfu1 function in the maturation of a subset of mitochondrial fe-s proteins
-
Navarro-Sastre A, Tort F, Stehling O, et al. (2011). A fatal mitochondrial disease is associated with defective NFU1 function in the maturation of a subset of mitochondrial Fe-S proteins. Am J Hum Genet 89: 656-67
-
(2011)
Am J Hum Genet
, vol.89
, pp. 656-667
-
-
Navarro-Sastre, A.1
Tort, F.2
Stehling, O.3
-
52
-
-
0035034465
-
Abortive assembly of succinate-ubiquinone reductase (complex ii) in a ferrochelatasedeficient mutant of escherichia coli
-
Nihei C, Nakayashiki T, Nakamura K, et al. (2001). Abortive assembly of succinate-ubiquinone reductase (complex II) in a ferrochelatasedeficient mutant of Escherichia coli. Mol Genet Genomics 265: 394-404
-
(2001)
Mol Genet Genomics
, vol.265
, pp. 394-404
-
-
Nihei, C.1
Nakayashiki, T.2
Nakamura, K.3
-
53
-
-
84866497654
-
Leukoencephalopathy with accumulated succinate is indicative of sdhaf1 related complex ii deficiency
-
Ohlenbusch A, Edvardson S, Skorpen J, et al. (2012). Leukoencephalopathy with accumulated succinate is indicative of SDHAF1 related complex II deficiency. Orphanet J Rare Dis 7: 69
-
(2012)
Orphanet J Rare Dis
, vol.7
, pp. 69
-
-
Ohlenbusch, A.1
Edvardson, S.2
Skorpen, J.3
-
54
-
-
0035907323
-
The quinone-binding sites of the saccharomyces cerevisiae succinate-ubiquinone oxidoreductase
-
Oyedotun KS, Lemire BD. (2001). The Quinone-binding sites of the Saccharomyces cerevisiae succinate-ubiquinone oxidoreductase. J Biol Chem 276: 16936-43
-
(2001)
J Biol Chem
, vol.276
, pp. 16936-16943
-
-
Oyedotun, K.S.1
Lemire, B.D.2
-
55
-
-
36549071581
-
The saccharomyces cerevisiae succinate dehydrogenase does not require heme for ubiquinone reduction
-
Oyedotun KS, Sit CS, Lemire BD. (2007). The Saccharomyces cerevisiae succinate dehydrogenase does not require heme for ubiquinone reduction. Biochim Biophys Acta 1767: 1436-45
-
(2007)
Biochim Biophys Acta
, vol.1767
, pp. 1436-1445
-
-
Oyedotun, K.S.1
Sit, C.S.2
Lemire, B.D.3
-
56
-
-
84869029429
-
Persulfide formation on mitochondrial cysteine desulfurase: Enzyme activation by a eukaryotespecific interacting protein and fe-s cluster synthesis
-
Pandey A, Golla R, Yoon H, et al. (2012). Persulfide formation on mitochondrial cysteine desulfurase: enzyme activation by a eukaryotespecific interacting protein and Fe-S cluster synthesis. Biochem J 448: 171-87
-
(2012)
Biochem J
, vol.448
, pp. 171-187
-
-
Pandey, A.1
Golla, R.2
Yoon, H.3
-
57
-
-
84891379841
-
Frataxin directly stimulates mitochondrial cysteine desulfurase by exposing substrate-binding sites, and a mutant fe-s cluster scaffold protein with frataxinbypassing ability acts similarly
-
Pandey A, Gordon DM, Pain J, et al. (2013). Frataxin directly stimulates mitochondrial cysteine desulfurase by exposing substrate-binding sites, and a mutant Fe-S cluster scaffold protein with frataxinbypassing ability acts similarly. J Biol Chem 288: 36773-86
-
(2013)
J Biol Chem
, vol.288
, pp. 36773-36786
-
-
Pandey, A.1
Gordon, D.M.2
Pain, J.3
-
58
-
-
79959715470
-
Sdha loss-of-function mutations in kit-pdgfra wild-Type gastrointestinal stromal tumors identified by massively parallel sequencing
-
Pantaleo MA, Astolfi A, Indio V. (2011). SDHA loss-of-function mutations in KIT-PDGFRA wild-Type gastrointestinal stromal tumors identified by massively parallel sequencing. J Natl Cancer Inst 103: 983-7
-
(2011)
J Natl Cancer Inst
, vol.103
, pp. 983-987
-
-
Pantaleo, M.A.1
Astolfi, A.2
Indio, V.3
-
59
-
-
84890807165
-
Analysis of all subunits, sdha, sdhb, sdhc, sdhd, of the succinate dehydrogenase complex in kit/pdgfra wild-Type gist
-
Pantaleo MA, Astolfi A, Urbini M, et al. (2014). Analysis of all subunits, SDHA, SDHB, SDHC, SDHD, of the succinate dehydrogenase complex in KIT/PDGFRA wild-Type GIST. Eur J Hum Genet 22: 32-9
-
(2014)
Eur J Hum Genet
, vol.22
, pp. 32-39
-
-
Pantaleo, M.A.1
Astolfi, A.2
Urbini, M.3
-
60
-
-
38549134313
-
Extra-Adrenal and adrenal pheochromocytomas associated with a germline sdhc mutation
-
Peczkowska M, Cascon A, Prejbisz A, et al. (2008). Extra-Adrenal and adrenal pheochromocytomas associated with a germline SDHC mutation. Nat Clin Practend Met 4: 111-15
-
(2008)
Nat Clin Practend Met
, vol.4
, pp. 111-115
-
-
Peczkowska, M.1
Cascon, A.2
Prejbisz, A.3
-
61
-
-
35348842764
-
Coa1 links the mss51 post-Translational function to cox1 cofactor insertion in cytochrome c oxidase assembly
-
Pierrel F, Bestwick ML, Cobine PA, et al. (2007). Coa1 links the Mss51 post-Translational function to Cox1 cofactor insertion in cytochrome c oxidase assembly. EMBO J 26: 4335-46
-
(2007)
EMBO J
, vol.26
, pp. 4335-4346
-
-
Pierrel, F.1
Bestwick, M.L.2
Cobine, P.A.3
-
62
-
-
49449095848
-
Coa2 is an assembly factor for yeast cytochrome c oxidase biogenesis that facilitates the maturation of cox1
-
Pierrel F, Khalimonchuk O, Cobine PA, et al. (2008). Coa2 is an assembly factor for yeast cytochrome c oxidase biogenesis that facilitates the maturation of Cox1. Mol Cell Biol 28: 4927-39
-
(2008)
Mol Cell Biol
, vol.28
, pp. 4927-4939
-
-
Pierrel, F.1
Khalimonchuk, O.2
Cobine, P.A.3
-
63
-
-
84867044598
-
Molecular organization, biochemical function, cellular role and evolution of nfua, an atypical fe-s carrier
-
Py B, Gerez C, Angelini S, et al. (2012). Molecular organization, biochemical function, cellular role and evolution of NfuA, an atypical Fe-S carrier. Mol Microbiol 86: 155-71
-
(2012)
Mol Microbiol
, vol.86
, pp. 155-171
-
-
Py, B.1
Gerez, C.2
Angelini, S.3
-
64
-
-
84896935583
-
The 2-oxoacid dehydrogenase complexes in mitochondria can produce superoxide/hydrogen peroxide at much higher rates than complex i
-
Quinlan CL, Goncalves RL, Hey-Mogensen M, et al. (2014). The 2-oxoacid dehydrogenase complexes in mitochondria can produce superoxide/hydrogen peroxide at much higher rates than complex I. J Biol Chem 289: 8312-25
-
(2014)
J Biol Chem
, vol.289
, pp. 8312-8325
-
-
Quinlan, C.L.1
Goncalves, R.L.2
Hey-Mogensen, M.3
-
65
-
-
0030048509
-
Covalent attachment of fad to the yeast succinate dehydrogenase flavoprotein requires import into mitochondria, presequence removal, and folding
-
Robinson KM, Lemire BD. (1996). Covalent attachment of FAD to the yeast succinate dehydrogenase flavoprotein requires import into mitochondria, presequence removal, and folding. J Biol Chem 271: 4055-60
-
(1996)
J Biol Chem
, vol.271
, pp. 4055-4060
-
-
Robinson, K.M.1
Lemire, B.D.2
-
66
-
-
0028298547
-
The covalent attachment of fad to the flavoprotein of saccharomyces cerevisiae succinate dehydrogenase is not necessary for import and assemblyinto mitochondria
-
Robinson KM, Rothery RA, Weiner JH, Lemire BD. (1994). The covalent attachment of FAD to the flavoprotein of Saccharomyces cerevisiae succinate dehydrogenase is not necessary for import and assemblYinto mitochondria. Eur J Biochem 222: 983-90
-
(1994)
Eur J Biochem
, vol.222
, pp. 983-990
-
-
Robinson, K.M.1
Rothery, R.A.2
Weiner, J.H.3
Lemire, B.D.4
-
67
-
-
0036226063
-
Grx5 is a mitochondrial glutaredoxin required for the activity of iron/sulfur enzymes
-
Rodriguez-Manzaneque MAT, Tamarit J, Bellí G, et al. (2002). Grx5 is a mitochondrial glutaredoxin required for the activity of iron/sulfur enzymes. Mol Biol Cell 13: 1109-21
-
(2002)
Mol Biol Cell
, vol.13
, pp. 1109-1121
-
-
Rodriguez-Manzaneque, M.A.T.1
Tamarit, J.2
Bellí, G.3
-
68
-
-
77952885778
-
Succinate dehydrogenase -Assembly, regulation and role in human disease
-
Rutter J, Winge DR, Schiffman JD. (2010). Succinate dehydrogenase -Assembly, regulation and role in human disease. Mitochondrion 10: 393-401
-
(2010)
Mitochondrion
, vol.10
, pp. 393-401
-
-
Rutter, J.1
Winge, D.R.2
Schiffman, J.D.3
-
69
-
-
0034666350
-
Molecular characterization of fmn1, the structural gene for the monofunctional flavokinase of saccharomyces cerevisiae
-
Santos MAA, Jiménez A, Revuelta J. (2000). Molecular characterization of FMN1, the structural gene for the monofunctional flavokinase of Saccharomyces cerevisiae. J Biol Chem 275: 28618-24
-
(2000)
J Biol Chem
, vol.275
, pp. 28618-28624
-
-
Santos, M.A.A.1
Jiménez, A.2
Revuelta, J.3
-
70
-
-
79551514731
-
Mammalian frataxin: An essential function for cellular viability through an interaction with a preformed iscu/nfs1/isd11 iron-sulfur assembly complex
-
Schmucker S, Martelli A, Colin F, et al. (2011). Mammalian frataxin: an essential function for cellular viability through an interaction with a preformed ISCU/NFS1/ISD11 iron-sulfur assembly complex. PLoS One 6: e16199
-
(2011)
PLoS One
, vol.6
, pp. e16199
-
-
Schmucker, S.1
Martelli, A.2
Colin, F.3
-
71
-
-
77955405138
-
Humans possess two mitochondrial ferredoxins, fdx1 and fdx2, with distinct roles in steroidogenesis, heme, and fe/s cluster biosynthesis
-
Sheftel AD, Stehling O, Pierik AJ, et al. (2010). Humans possess two mitochondrial ferredoxins, Fdx1 and Fdx2, with distinct roles in steroidogenesis, heme, and Fe/S cluster biosynthesis. Proc Natl Acad Sci USA 107: 11775-80
-
(2010)
Proc Natl Acad Sci USA
, vol.107
, pp. 11775-11780
-
-
Sheftel, A.D.1
Stehling, O.2
Pierik, A.J.3
-
72
-
-
84859400502
-
The human mitochondrial isca1, isca2, and iba57 proteins are required for [4fe-4s] protein maturation
-
Sheftel AD, Wilbrecht C, Stehling O, et al. (2012). The human mitochondrial ISCA1, ISCA2, and IBA57 proteins are required for [4Fe-4S] protein maturation. Mol Biol Cell 23: 1157-66
-
(2012)
Mol Biol Cell
, vol.23
, pp. 1157-1166
-
-
Sheftel, A.D.1
Wilbrecht, C.2
Stehling, O.3
-
73
-
-
33847100058
-
The role of sdh4p tyr-89 in ubiquinone reduction by the saccharomyces cerevisiae succinate dehydrogenase
-
Silkin Y, Oyedotun KS, Lemire BD. (2007). The role of Sdh4p Tyr-89 in ubiquinone reduction by the Saccharomyces cerevisiae succinate dehydrogenase. Biochim Biophys Acta 1767: 143-50
-
(2007)
Biochim Biophys Acta
, vol.1767
, pp. 143-150
-
-
Silkin, Y.1
Oyedotun, K.S.2
Lemire, B.D.3
-
74
-
-
21244503033
-
Crystal structure of mitochondrial respiratory membrane protein complex II
-
Sun F, Huo X, Zhai Y, et al. (2005). Crystal structure of mitochondrial respiratory membrane protein complex II. Cell 121: 1043-57
-
(2005)
Cell
, vol.121
, pp. 1043-1057
-
-
Sun, F.1
Huo, X.2
Zhai, Y.3
-
75
-
-
77949913668
-
Mitochondrial localization of human fad synthetase isoform 1
-
Torchetti EM, Brizio C, Colella M, et al. (2010). Mitochondrial localization of human FAD synthetase isoform 1. Mitochondrion 10: 263-73
-
(2010)
Mitochondrion
, vol.10
, pp. 263-273
-
-
Torchetti, E.M.1
Brizio, C.2
Colella, M.3
-
76
-
-
78049305276
-
Human frataxin is an allosteric switch that activates the fe-s cluster biosynthetic complex
-
Tsai CL, Barondeau DP. (2010). Human frataxin is an allosteric switch that activates the Fe-S cluster biosynthetic complex. Biochemistry 49: 9132-9
-
(2010)
Biochemistry
, vol.49
, pp. 9132-9139
-
-
Tsai, C.L.1
Barondeau, D.P.2
-
77
-
-
0029984499
-
Flx1 codes for a carrier protein involved in maintaining a proper balance of flavin nucleotides in yeast mitochondria
-
Tzagoloff A, Jang J, Glerum DM, Wu M. (1996). FLX1 codes for a carrier protein involved in maintaining a proper balance of flavin nucleotides in yeast mitochondria. J Biol Chem 271: 7392-7
-
(1996)
J Biol Chem
, vol.271
, pp. 7392-7397
-
-
Tzagoloff, A.1
Jang, J.2
Glerum, D.M.3
Wu, M.4
-
78
-
-
84879562737
-
The mitochondrial hsp70 chaperone ssq1 facilitates fe/s cluster transfer from isu1 to grx5 by complex formation
-
Uzarska MA, Dutkiewicz R, Freibert SA, et al. (2013). The mitochondrial Hsp70 chaperone Ssq1 facilitates Fe/S cluster transfer from Isu1 to Grx5 by complex formation. Mol Biol Cell 24: 1830-41
-
(2013)
Mol Biol Cell
, vol.24
, pp. 1830-1841
-
-
Uzarska, M.A.1
Dutkiewicz, R.2
Freibert, S.A.3
-
79
-
-
84905821769
-
Sdhaf4 promotes mitochondrial succinate dehydrogenase activity and prevents neurodegeneration
-
Van Vranken JG, Bricker DK, Dephoure N, et al. (2014). SDHAF4 promotes mitochondrial succinate dehydrogenase activity and prevents neurodegeneration. Cell Metab 20: 241-52
-
(2014)
Cell Metab
, vol.20
, pp. 241-252
-
-
Van Vranken, J.G.1
Bricker, D.K.2
Dephoure, N.3
-
80
-
-
9144249602
-
Early-onset renal cell carcinoma as a novel extraparaganglial component of sdhbassociated heritable paraganglioma
-
Vanharanta S, Buchta M, Mcwhinney SR, et al. (2004). Early-onset renal cell carcinoma as a novel extraparaganglial component of SDHBassociated heritable paraganglioma. Am J Hum Genet 74: 153-9
-
(2004)
Am J Hum Genet
, vol.74
, pp. 153-159
-
-
Vanharanta, S.1
Buchta, M.2
McWhinney, S.R.3
-
81
-
-
0028950476
-
Cloning and characterization of fad1, the structural gene for flavin adenine dinucleotide synthetase of saccharomyces cerevisiae
-
Wu M, Repetto B, Glerum DM, Tzagoloff A. (1995). Cloning and characterization of FAD1, the structural gene for flavin adenine dinucleotide synthetase of Saccharomyces cerevisiae. Mol Cell Biol 15: 264-71
-
(1995)
Mol Cell Biol
, vol.15
, pp. 264-271
-
-
Wu, M.1
Repetto, B.2
Glerum, D.M.3
Tzagoloff, A.4
-
82
-
-
0348150715
-
Architecture of succinate dehydrogenase and reactive oxygen species generation
-
Yankovskaya V, Horsefield R, Tornroth S, et al. (2003). Architecture of succinate dehydrogenase and reactive oxygen species generation. Science 299: 700-4
-
(2003)
Science
, vol.299
, pp. 700-704
-
-
Yankovskaya, V.1
Horsefield, R.2
Tornroth, S.3
|