Clinical and molecular findings in children with complex I deficiency
Open accessAbstract
Isolated complex I deficiency, the most frequent OXPHOS disorder in infants and children, is genetically heterogeneous. Mutations have been found in seven mitochondrial DNA (mtDNA) and eight nuclear DNA encoded subunits, respectively, but in most of the cases the genetic basis of the biochemical defect is unknown. We analyzed the entire mtDNA and 11 nuclear encoded complex I subunits in 23 isolated complex I-deficient children, classified into five clinical groups: Leigh syndrome, progressive leukoencephalopathy, neonatal cardiomyopathy, severe infantile lactic acidosis, and a miscellaneous group of unspecified encephalomyopathies. A genetic definition was reached in eight patients (35%). Mutations in mtDNA were found in six out of eight children with Leigh syndrome, indicating a prevalent association between this phenotype and abnormalities in ND genes. In two patients with leukoencephalopathy, homozygous mutations were detected in two different nuclear-encoded complex I genes, including a novel transition in NDUFS1 subunit. In addition to these, a child affected by mitochondrial encephalomyopathy had heterozygous mutations in NDUFA8 and NDUFS2 genes, while another child with neonatal cardiomyopathy had a complex rearrangement in a single NDUFS7 allele. The latter cases suggest the possibility of unconventional patterns of inheritance in complex I defects.
Integrity signals
Registry
Cites
Statistics
Claims the paper makes
Claims other papers make about this one
Each is checked against this paper’s own text: does it say what the citing paper says it does?
PARP inhibitors lessen genetic mitochondrial metabolism defects in human fibroblasts.
“Furthermore, PARP inhibitors reverse mitochondrial defects in primary myotubes of obese humans and attenuate genetic defects of mitochondrial metabolism in human fibroblasts and C. elegans.”
From Pharmacological Inhibition of poly(ADP-ribose) polymerases improves fitness and mitochondrial function in skeletal muscle (2014) · cites this paper as Bugiani et al., 2004
NDUFS1 mutant fibroblasts have a mutation that lowers complex I activity by 87%.
“To test whether these observations could also apply in human models, we used skin fibroblasts from a patient with a mutation in the nuclear NDUFS1 gene, which reduced complex I activity by 87% (Bugiani et al., 2004).”
From Pharmacological Inhibition of poly(ADP-ribose) polymerases improves fitness and mitochondrial function in skeletal muscle (2014) · cites this paper as Bugiani et al., 2004
Cites
PROTEIN MEASUREMENT WITH THE FOLIN PHENOL REAGENT
Lowry, O. H., Rosebrough, N., Farr, A. L., & Randall, R. (1951) · Journal of Biological Chemistry · doi:10.1016/s0021-9258(19)52451-6 · Cited by 315,771 · see citing works
Mitochondrial DNA mutation associated with Leber’s hereditary optic neuropathy
Wallace, D. C., Singh, G., Lott, M. T., Hodge, J. A., Schurr, T. G., Lezza, A. M. S., Elsas, L. J., & Nikoskelainen, E. K. (1988) · Science · doi:10.1126/science.3201231 · Cited by 2,440 · see citing works
Srere, P. A. (1969) · Methods in Enzymology · doi:10.1016/0076-6879(69)13005-0 · Cited by 2,005 · see citing works
[45] Cytochrome oxidase from beef heart mitochondria
Wharton, D. C., & Tzagoloff, A. (1967) · Methods in enzymology on CD-ROM/Methods in enzymology · doi:10.1016/0076-6879(67)10048-7 · Cited by 1,266 · see citing works
Leigh syndrome: Clinical features and biochemical and DNA abnormalities
Rahman, S., Blok, R. B., Dahl, H. ‐. M., Danks, D. M., Kirby, D. M., Chow, C. W., Christodoulou, J., & Thorburn, D. R. (1996) · Annals of Neurology · doi:10.1002/ana.410390311 · Cited by 776 · see citing works
Mitochondria: a practical approach.
Darley-Usmar, V. M., RICKWOOD, D., & Wilson, M. T. (1987) · IRL Press eBooks · Cited by 410 · see citing works
Analysis of the Subunit Composition of Complex I from Bovine Heart Mitochondria*S
Carroll, J., Fearnley, I. M., Shannon, R. J., Hirst, J., & Walker, J. E. (2003) · Molecular & Cellular Proteomics · doi:10.1074/mcp.m300014-mcp200 · Cited by 362 · see citing works
Fearnley, I. M., & Walker, J. E. (1992) · Biochimica et Biophysica Acta (BBA) - Bioenergetics · doi:10.1016/0005-2728(92)90001-i · Cited by 308 · see citing works
Mitochondria: A practical approach
Jacobs, H. T. (1988) · Trends in Genetics · doi:10.1016/0168-9525(88)90164-3 · Cited by 292 · see citing works
Mutant NDUFV1 subunit of mitochondrial complex I causes leukodystrophy and myoclonic epilepsy
Schuelke, M., Smeitink, J. A. M., Mariman, E. C. M., Loeffen, J. L. C., Plecko, B., Trijbels, F. J. M., Stöckler‐Ipsiroglu, S., & van den Heuvel, L. (1999) · Nature Genetics · doi:10.1038/6772 · Cited by 270 · see citing works
Respiratory chain complex I deficiency
Kirby, D. M., Crawford, M., Cleary, M., Dahl, H. ‐. M., Dennett, X., & Thorburn, D. R. (1999) · Neurology · doi:10.1212/wnl.52.6.1255 · Cited by 266 · see citing works
Bénit, P., Chrétien, D., Kadhom, N., de Lonlay, P., Cormier‐Daire, V., Cabral, A., Peudenier, S., Rustin, P., Münnich, A., & Rötig, A. (2001) · The American Journal of Human Genetics · doi:10.1086/320603 · Cited by 261 · see citing works
Ugalde, C. (2004) · Human Molecular Genetics · doi:10.1093/hmg/ddh071 · Cited by 216 · see citing works
Tiranti, V., Chariot, P., Carella, F., Toscano, A., Soliveri, P., Girlanda, P., Carrara, F., Fratta, G. M., Reid, F. M., Mariotti, C., et al. (1995) · Human Molecular Genetics · doi:10.1093/hmg/4.8.1421 · Cited by 208 · see citing works
Self-citationAdult-derived neural precursors transplanted into multiple regions in the adult brain
Herrera, D. G., García‐Verdugo, J. M., & Álvarez-Buylla, A. (1999) · Annals of Neurology · doi:10.1002/1531-8249(199912)46:6 · Cited by 204 · see citing works
McFarland, R., Kirby, D. M., Fowler, K. J., Ohtake, A., Ryan, M. T., Amor, D. J., Fletcher, J. M., Dixon, J. W., Collins, F. A., Turnbull, D. M., et al. (2003) · Annals of Neurology · doi:10.1002/ana.10787 · Cited by 180 · see citing works
Identification of a Novel Mutation in the mtDNA ND5 Gene Associated with MELAS
Santorelli, F. M., Tanji, K., Kulikova, R., Shanske, S., Vilarinho, L., Hays, A. P., & DiMauro, S. (1997) · Biochemical and Biophysical Research Communications · doi:10.1006/bbrc.1997.7167 · Cited by 170 · see citing works
Quantitative neuropathological changes in presymptomatic Huntington's disease
Gómez‐Tortosa, E., MacDonald, M. E., Friend, J. C., Taylor, S. A., Weiler, L., Cupples, L. A., Srinidhi, J., Gusella, J. F., Bird, E. D., Vonsattel, J., et al. (2001) · Annals of Neurology · doi:10.1002/1531-8249(200101)49:1 · Cited by 170 · see citing works
Self-citationChinnery, P. F., Brown, D. T., Andrews, R. M., Kler, R. S., Riordan‐Eva, P., Lindley, J., Applegarth, D. A., Turnbull, D. M., & Howell, N. (2001) · Brain · doi:10.1093/brain/124.1.209 · Cited by 166 · see citing works
Chol, M., Lebon, S., Bénit, P., Chrétien, D., de Lonlay, P., Goldenberg, A., Odent, S., Hertz‐Pannier, L., Vincent-Delorme, C., Cormier‐Daire, V., et al. (2003) · Journal of Medical Genetics · doi:10.1136/jmg.40.3.188 · Cited by 150 · see citing works
Zheng, X., Shoffner, J. M., Voljavec, A. S., & Wallace, D. C. (1990) · Biochimica et Biophysica Acta (BBA) - Bioenergetics · doi:10.1016/0005-2728(90)90118-n · Cited by 145 · see citing works
Characterization of SURF-1 Expression and Surf-1p Function in Normal and Disease Conditions
Tiranti, V., Galimberti, C., Nijtmans, L. G. J., Bovolenta, S., Perini, M. P., & Zeviani, M. (1999) · Human Molecular Genetics · doi:10.1093/hmg/8.13.2533 · Cited by 134 · see citing works
Self-citationEsposti, M. D., Carelli, V., Ghelli, A. M., Ratta, M., Crimi, M., Sangiorgi, S., Montagna, P., Lenaz, G., Lugaresi, E., & Cortelli, P. (1994) · FEBS Letters · doi:10.1016/0014-5793(94)00971-6 · Cited by 123 · see citing works
Recurrent de novo mitochondrial DNA mutations in respiratory chain deficiency
Lebon, S., Chol, M., Bénit, P., Mugnier, C., Chrétien, D., Giurgea, I., Kern, I., Girardin, E. P., Hertz‐Pannier, L., de Lonlay, P., et al. (2003) · Journal of Medical Genetics · doi:10.1136/jmg.40.12.896 · Cited by 123 · see citing works
Low mutant load of mitochondrial DNA G13513A mutation can cause Leigh's disease
Kirby, D. M., Boneh, A., Chow, C. W., Ohtake, A., Ryan, M. T., Thyagarajan, D. E., & Thorburn, D. R. (2003) · Annals of Neurology · doi:10.1002/ana.10687 · Cited by 116 · see citing works
Ugalde, C., Triepels, R. H., Coenen, M. J. H., van den Heuvel, L. P., Smeets, R., Uusimaa, J., Briones, P., Campistol, J., Majamaa, K., Smeitink, J. A. M., et al. (2003) · Annals of Neurology · doi:10.1002/ana.10734 · Cited by 115 · see citing works
MR findings in Leigh syndrome with COX deficiency and SURF-1 mutations.
Farina, L., Chiapparini, L., Uziel, G., Bugiani, M., Zeviani, M., & Savoiardo, M. (2002) · PubMed Central · Cited by 98 · see citing works
Self-citationRavn, K., Wibrand, F., Hansen, F. J., Horn, N., Rosenberg, T., & Schwartz, M. (2001) · European Journal of Human Genetics · doi:10.1038/sj.ejhg.5200712 · Cited by 98 · see citing works
Taylor, R. W., Kler, R. S., Hayes, C. M., Smith, P. E. M., & Turnbull, D. M. (2001) · Annals of Neurology · doi:10.1002/ana.1084 · Cited by 97 · see citing works
Antoine, J.-C. G., Cinotti, L., Tilikete, C., Bouhour, F., Camdessanch�, J. P., Confavreux, C., Vighetto, A., Renault‐Mannel, V., Michel, D., & Honnorat, J. (2000) · Annals of Neurology · doi:10.1002/1531-8249(200007)48:1 · Cited by 86 · see citing works
Maternal inheritance and the evaluation of oxidative phosphorylation diseases
Shoffner, J. M. (1996) · The Lancet · doi:10.1016/s0140-6736(96)09138-6 · Cited by 83 · see citing works
Tiranti, V., Munaro, M., Sandonà, D., Lamantea, E., Rimoldi, M., DiDonato, S., Bisson, R., & Zeviani, M. (1995) · Human Molecular Genetics · doi:10.1093/hmg/4.11.2017 · Cited by 82 · see citing works
Self-citationCerebral White Matter Involvement in Children with Mitochondrial Encephalopathies
Moroni, I., Bugiani, M., Bizzi, A., Castelli, G., Lamantea, E., & Uziel, G. (2002) · Neuropediatrics · doi:10.1055/s-2002-32372 · Cited by 79 · see citing works
Self-citationA New Mitochondrial DNA Mutation in ND3 Gene Causing Severe Leigh Syndrome with Early Lethality
Crimi, M., Papadimitriou, A., Galbiati, S., Palamidou, P., Fortunato, F., Bordoni, A., Papandreou, U., Papadimitriou, D. A., Hadjigeorgiou, G. M., Drogari, E., et al. (2004) · Pediatric Research · doi:10.1203/01.pdr.0000117844.73436.68 · Cited by 78 · see citing works
Gray, K. A., Dutton, P. L., & Daldal, F. (1994) · Biochemistry · doi:10.1021/bi00169a014 · Cited by 78 · see citing works
Bilateral striatal necrosis associated with a novel mutation in the mitochondrial ND6 gene
Solano, A., Roig-Quilis, M., Vives-Bauzá, C., Hernandez‐Peña, J., García‐Arumí, E., Playán, A., López-Pérez, M. J., Andreu, A. L., & Montoya, J. (2003) · Annals of Neurology · doi:10.1002/ana.10682 · Cited by 74 · see citing works
Sudo, A., Honzawa, S., Nonaka, I., & Goto, Y. (2004) · Journal of Human Genetics · doi:10.1007/s10038-003-0116-1 · Cited by 67 · see citing works
Pitkänen, S., Feigenbaum, A., Laframboise, R., & Robinson, B. H. (1996) · Journal of Inherited Metabolic Disease · doi:10.1007/bf01799845 · Cited by 64 · see citing works
A novel mtDNA C11777A mutation in Leigh syndrome
Komaki, H., Akanuma, J., Iwata, H., Takahashi, T., Mashima, Y., Nonaka, I., & Goto, Y.-I. (2003) · Mitochondrion · doi:10.1016/s1567-7249(03)00003-5 · Cited by 50 · see citing works
Identification of P gene mutations in individuals with oculocutaneous albinism in sub-Saharan Africa
Kerr, R. A., Stevens, G., Manga, P., Salm, S. N., John, P. R., Haw, T., & Ramsay, M. (2000) · Human Mutation · doi:10.1002/(sici)1098-1004(200002)15:2 · Cited by 50 · see citing works
Recurrent brain hematomas in MELAS associated with an ND5 gene mitochondrial mutation
Pénisson-Besnier, I., Reynier, P., Asfar, P., Douay, O., Sortais, A., Dubas, F., Emile, J., & Malthièry, Y. (2000) · Neurology · doi:10.1212/wnl.55.2.317 · Cited by 45 · see citing works
Late-onset encephalopathy associated with a C11777A mutation of mitochondrial DNA
Deschauer, M., Bamberg, C., Claus, D., Zierz, S., Turnbull, D. M., & Taylor, R. W. (2003) · Neurology · doi:10.1212/01.wnl.0000055869.99975.4b · Cited by 33 · see citing works
Price (1999) · doi:10.1002/1531-8249(199903)45:3
Flagged first, then by citations
1 of the 44 references couldn’t be matched to a known paper.
Cited by
Sorted by citations
The Mitochondrial Proteome and Human Disease
Calvo, S. E., & Mootha, V. K. (2010) · Annual Review of Genomics and Human Genetics · doi:10.1146/annurev-genom-082509-141720 · Cited by 590 · see citing works
Varanita, T., Soriano, M. E., Romanello, V., Zaglia, T., Quintana-Cabrera, R., Semenzato, M., Menabò, R., Costa, V., Civiletto, G., Pesce, P., et al. (2015) · Cell Metabolism · doi:10.1016/j.cmet.2015.05.007 · Cited by 460 · see citing works
Self-citationMitochondrial respiratory complex I: Structure, function and implication in human diseases
Sharma, L., Lu, J., & Bai, Y. (2009) · Current Medicinal Chemistry · doi:10.2174/092986709787846578 · Cited by 438 · see citing works
Spinazzola, A., Viscomi, C., Fernandez-Vizarra, E., Carrara, F., D'Adamo, P., Calvo, S., Marsano, R. M., Donnini, C., Weiher, H., Strisciuglio, P., et al. (2006) · Nature Genetics · doi:10.1038/ng1765 · Cited by 427 · see citing works
Self-citationTiranti, V., Viscomi, C. F., Hildebrandt, T. M., Meo, I. D., Mineri, R., Tiveron, C., Levitt, M. D., Prelle, A. C., Fagiolari, G., Rimoldi, M., et al. (2009) · Nature Medicine · doi:10.1038/nm.1907 · Cited by 404 · see citing works
Self-citationMigliavacca, E., Tay, S. K. H., Patel, H. P., Sonntag, T., Civiletto, G., McFarlane, C., Forrester, T., Barton, S. J., Leow, M. K., Antoun, E., et al. (2019) · Nature Communications · doi:10.1038/s41467-019-13694-1 · Cited by 365 · see citing works
Calvo, S. E., Tucker, E. J., Compton, A. G., Kirby, D. M., Crawford, G., Burtt, N. P., Rivas, M. A., Guiducci, C., Bruno, D. L., Goldberger, O., et al. (2010) · Nature Genetics · doi:10.1038/ng.659 · Cited by 362 · see citing works
NAD+-Dependent Activation of Sirt1 Corrects the Phenotype in a Mouse Model of Mitochondrial Disease
Cerutti, R., Pirinen, E., Lamperti, C., Marchet, S., Sauve, A. A., Li, W., Leoni, V., Schon, E. A., Dantzer, F., Auwerx, J., et al. (2014) · Cell Metabolism · doi:10.1016/j.cmet.2014.04.001 · Cited by 343 · see citing works
Self-citationComplex I deficiency: clinical features, biochemistry and molecular genetics
Fassone, E., & Rahman, S. (2012) · Journal of Medical Genetics · doi:10.1136/jmedgenet-2012-101159 · Cited by 316 · see citing works
Transcription Factor EB Controls Metabolic Flexibility during Exercise
Mansueto, G., Armani, A., Viscomi, C., D’Orsi, L., De Cegli, R., Polishchuk, E. V., Lamperti, C., Di Meo, I., Romanello, V., Marchet, S., et al. (2017) · Cell Metabolism · doi:10.1016/j.cmet.2016.11.003 · Cited by 314 · see citing works
Self-citationMitochondrial disorders of the OXPHOS system
Fernández‐Vizarra, E., & Zeviani, M. (2020) · FEBS Letters · doi:10.1002/1873-3468.13995 · Cited by 304 · see citing works
Self-citationInfantile hepatocerebral syndromes associated with mutations in the mitochondrial DNA polymerase- A
Ferrari, G. (2005) · Brain · doi:10.1093/brain/awh410 · Cited by 292 · see citing works
In Vivo Correction of COX Deficiency by Activation of the AMPK/PGC-1α Axis
Viscomi, C. F., Bottani, E., Civiletto, G., Cerutti, R., Moggio, M. G., Fagiolari, G., Schon, E. A., Lamperti, C., & Zeviani, M. (2011) · Cell Metabolism · doi:10.1016/j.cmet.2011.04.011 · Cited by 291 · see citing works
Self-citationMitochondrial complex I: structure, function and pathology
Janssen, R. J. R. J., Nijtmans, L. G., Heuvel, L. P. V. D., & Smeitink, J. A. M. (2006) · Journal of Inherited Metabolic Disease · doi:10.1007/s10545-006-0362-4 · Cited by 276 · see citing works
Pirinen, E., Cantó, C., Jo, Y. S., Morato, L., Zhang, H., Menzies, K. J., Williams, E. G., Mouchiroud, L., Moullan, N., Hagberg, C., et al. (2014) · Cell Metabolism · doi:10.1016/j.cmet.2014.04.002 · Cited by 254 · see citing works
“Myoblasts from obese patients and NDUFS1mutant fibroblasts were provided by Drs. P. Schrauwen (Timmers et al., 2012) and M. Zeviani (Bugiani et al., 2004).”
BackgroundMethodSelf-citationMitochondrial Structure and Bioenergetics in Normal and Disease Conditions
Protasoni, M., & Zeviani, M. (2021) · International Journal of Molecular Sciences · doi:10.3390/ijms22020586 · Cited by 223 · see citing works
Self-citationOpa1 Overexpression Ameliorates the Phenotype of Two Mitochondrial Disease Mouse Models
Civiletto, G., Varanita, T., Cerutti, R., Gorletta, T., Barbaro, S., Marchet, S., Lamperti, C., Viscomi, C., Scorrano, L., & Zeviani, M. (2015) · Cell Metabolism · doi:10.1016/j.cmet.2015.04.016 · Cited by 216 · see citing works
Self-citationFernández-Vizarra, E., Tiranti, V., & Zeviani, M. (2009) · Biochimica et Biophysica Acta (BBA) - Molecular Cell Research · doi:10.1016/j.bbamcr.2008.05.028 · Cited by 214 · see citing works
Self-citationSteenweg, M. E., Ghezzi, D., Haack, T. B., Abbink, T. E. M., Martinelli, D., van Berkel, C. G. M., Bley, A. E., Diogo, L. M., Grillo, E., Naudé, J. T. W., et al. (2012) · Brain · doi:10.1093/brain/aws070 · Cited by 216 · see citing works
Self-citationAssembly of mitochondrial complex I and defects in disease
Lazarou, M., Thorburn, D. R., Ryan, M. T., & McKenzie, M. (2008) · Biochimica et Biophysica Acta (BBA) - Molecular Cell Research · doi:10.1016/j.bbamcr.2008.04.015 · Cited by 212 · see citing works
Valente, L., Tiranti, V., Marsano, R. M., Malfatti, E., Fernández‐Vizarra, E., Donnini, C., Mereghetti, P., GIOIA, L. D., Burlina, A., Castellan, C. C., et al. (2006) · The American Journal of Human Genetics · doi:10.1086/510559 · Cited by 206 · see citing works
Self-citationMassa, V., Fernández‐Vizarra, E., AlShahwan, S., Bakhsh, E., Goffrini, P., Ferrero, I., Mereghetti, P., D’Adamo, P., Gasparini, P., & Zeviani, M. (2008) · The American Journal of Human Genetics · doi:10.1016/j.ajhg.2008.05.002 · Cited by 198 · see citing works
Self-citationHarel, T., Yoon, W. H., Garone, C., Gu, S., Coban‐Akdemir, Z., Eldomery, M. K., Posey, J. E., Jhangiani, S. N., Rosenfeld, J. A., Cho, M. T., et al. (2016) · The American Journal of Human Genetics · doi:10.1016/j.ajhg.2016.08.007 · Cited by 191 · see citing works
Self-citationClinical and molecular features of mitochondrial DNA depletion syndromes
Spinazzola, A., Invernizzi, F., Carrara, F., Lamantea, E., Donati, A., DiRocco, M., Giordano, I., Meznarič, M., Baruffini, E., Ferrero, I., et al. (2008) · Journal of Inherited Metabolic Disease · doi:10.1007/s10545-008-1038-z · Cited by 184 · see citing works
Self-citationFernández‐Vizarra, E., Bugiani, M., Goffrini, P., Carrara, F., Farina, L., Procopio, E., Donati, A., Uziel, G., Ferrero, I., & Zeviani, M. (2007) · Human Molecular Genetics · doi:10.1093/hmg/ddm072 · Cited by 181 · see citing works
Self-citationGene-specific mitochondria dysfunctions in human TARDBP and C9ORF72 fibroblasts
Onesto, E., Colombrita, C., Gumina, V., Borghi, M. O., Dusi, S., Doretti, A., Fagiolari, G., Invernizzi, F., Moggio, M. G., Tiranti, V., et al. (2016) · Acta Neuropathologica Communications · doi:10.1186/s40478-016-0316-5 · Cited by 181 · see citing works
Self-citationGai, X., Ghezzi, D., Johnson, M. A., Biagosch, C., Shamseldin, H. E., Haack, T. B., Reyes, A., Tsukikawa, M., Sheldon, C. A., Srinivasan, S., et al. (2013) · The American Journal of Human Genetics · doi:10.1016/j.ajhg.2013.07.016 · Cited by 180 · see citing works
Self-citationGhezzi, D., Arzuffi, P., Zordan, M. A., Da‐Ré, C., Lamperti, C., Benna, C., D’Adamo, P., Diodato, D., Costa, R., Mariotti, C., et al. (2011) · Nature Genetics · doi:10.1038/ng.761 · Cited by 172 · see citing works
Self-citationMammalian Mitochondrial Complex I Structure and Disease-Causing Mutations
Fiedorczuk, K., & Sazanov, L. A. (2018) · Trends in Cell Biology · doi:10.1016/j.tcb.2018.06.006 · Cited by 161 · see citing works
Combined defects in oxidative phosphorylation and fatty acid β-oxidation in mitochondrial disease
Nsiah-Sefaa, A., & McKenzie, M. (2016) · Bioscience Reports · doi:10.1042/bsr20150295 · Cited by 158 · see citing works
Iuso, A., Scacco, S., Piccoli, C., Bellomo, F., Petruzzella, V., Trentadue, R., Minuto, M. N., Ripoli, M. C., Capitanio, N., Zeviani, M., et al. (2006) · Journal of Biological Chemistry · doi:10.1074/jbc.m513387200 · Cited by 149 · see citing works
Self-citationFernández‐Vizarra, E., Berardinelli, A. L., Valente, L., Tiranti, V., & Zeviani, M. (2006) · Journal of Medical Genetics · doi:10.1136/jmg.2006.045252 · Cited by 148 · see citing works
Self-citationAssembly Factors of Human Mitochondrial Respiratory Chain Complexes: Physiology and Pathophysiology
Ghezzi, D., & Zeviani, M. (2012) · Advances in experimental medicine and biology · doi:10.1007/978-1-4614-3573-0_4 · Cited by 144 · see citing works
Self-citationNovel mutations of ND genes in complex I deficiency associated with mitochondrial encephalopathy
Malfatti, E., Bugiani, M., Invernizzi, F., de Souza, C. F. M., Farina, L., Carrara, F., Lamantea, E., Antozzi, C. G., Confalonieri, P. A., Sanseverino, M. T. V., et al. (2007) · Brain · doi:10.1093/brain/awm114 · Cited by 145 · see citing works
Self-citationX‐linked NDUFA1 gene mutations associated with mitochondrial encephalomyopathy
Fernández-Moreira, D., Ugalde, C., Smeets, R., Rodenburg, R. J., López‐Laso, E., Ruiz-Falcó, M. L., Briones, P., Martín, M. A., Smeitink, J. A. M., & Arenas, J. (2007) · Annals of Neurology · doi:10.1002/ana.21036 · Cited by 141 · see citing works
Koene, S., Rodenburg, R. J., van der Knaap, M. S., Willemsen, M. A. A. P., Sperl, W., Laugel, V., Østergaard, E., Tarnopolsky, M. A., Martín, M. A., Nesbitt, V., et al. (2012) · Journal of Inherited Metabolic Disease · doi:10.1007/s10545-012-9492-z · Cited by 132 · see citing works
Respiratory chain complex I deficiency caused by mitochondrial DNA mutations
Swalwell, H., Kirby, D. M., Blakely, E. L., Mitchell, A., Salemi, R., Sugiana, C., Compton, A. G., Tucker, E. J., Ke, B., Lamont, P. J., et al. (2011) · European Journal of Human Genetics · doi:10.1038/ejhg.2011.18 · Cited by 128 · see citing works
Chen, Y., Chen, C., Zhang, L., Green‐Church, K. B., & Zweier, J. L. (2005) · Journal of Biological Chemistry · doi:10.1074/jbc.m503936200 · Cited by 127 · see citing works
Gerber, S., Charif, M., Chevrollier, A., Chaumette, T., Angebault, C., Kane, M. S., Paris, A., Alban, J., Quilès, M., Delettre, C., et al. (2017) · Brain · doi:10.1093/brain/awx219 · Cited by 125 · see citing works
Civiletto, G., Doğan, Ş. A., Cerutti, R., Fagiolari, G., Moggio, M. G., Lamperti, C., Benincá, C., Viscomi, C. F., & Zeviani, M. (2018) · EMBO Molecular Medicine · doi:10.15252/emmm.201708799 · Cited by 116 · see citing works
Self-citationGhezzi, D., Saada, A., D’Adamo, P., Fernández‐Vizarra, E., Gasparini, P., Tiranti, V., Elpeleg, O. N., & Zeviani, M. (2008) · The American Journal of Human Genetics · doi:10.1016/j.ajhg.2008.08.009 · Cited by 115 · see citing works
Self-citationBrunetti, D., Dusi, S., Morbin, M., Uggetti, A., Moda, F., D’Amato, I., Giordano, C., d’Amati, G., Cozzi, A., Levi, S., et al. (2012) · Human Molecular Genetics · doi:10.1093/hmg/dds380 · Cited by 110 · see citing works
Laar, V. S. V., Dukes, A. A., Cascio, M., & Hastings, T. G. (2007) · Neurobiology of Disease · doi:10.1016/j.nbd.2007.11.007 · Cited by 109 · see citing works
Human diseases associated with defects in assembly of OXPHOS complexes
Ghezzi, D., & Zeviani, M. (2018) · Essays in Biochemistry · doi:10.1042/ebc20170099 · Cited by 106 · see citing works
Self-citationEngineering RNA endonucleases with customized sequence specificities
Choudhury, R., Tsai, Y., Dominguez, D., Wang, Y., & Wang, Z. (2012) · Nature Communications · doi:10.1038/ncomms2154 · Cited by 106 · see citing works
Identification of novel mutations in five patients with mitochondrial encephalomyopathy
Valente, L., Piga, D., Lamantea, E., Carrara, F., Uziel, G., Cudia, P., Zani, A., Farina, L., Morandi, L. O., Mora, M., et al. (2008) · Biochimica et Biophysica Acta (BBA) - Bioenergetics · doi:10.1016/j.bbabio.2008.10.001 · Cited by 102 · see citing works
Self-citationVARS2andTARS2Mutations in Patients with Mitochondrial Encephalomyopathies
Diodato, D., Melchionda, L., Haack, T. B., Dallabona, C., Baruffini, E., Donnini, C., Granata, T., Ragona, F., Balestri, P., Margollicci, M. A., et al. (2014) · Human Mutation · doi:10.1002/humu.22590 · Cited by 99 · see citing works
Self-citationPerturbed Redox Signaling Exacerbates a Mitochondrial Myopathy
Doğan, Ş. A., Cerutti, R., Benincá, C., Brea‐Calvo, G., Jacobs, H. T., Zeviani, M., Szibor, M., & Viscomi, C. F. (2018) · Cell Metabolism · doi:10.1016/j.cmet.2018.07.012 · Cited by 98 · see citing works
Self-citationLeigh Syndrome: A Tale of Two Genomes
Bakare, A. B., Lesnefsky, E. J., & Iyer, S. (2021) · Frontiers in Physiology · doi:10.3389/fphys.2021.693734 · Cited by 101 · see citing works
Meo, I. D., Fagiolari, G., Prelle, A. C., Viscomi, C. F., Zeviani, M., & Tiranti, V. (2010) · Antioxidants and Redox Signaling · doi:10.1089/ars.2010.3520 · Cited by 93 · see citing works
Self-citationMicroscale oxygraphy reveals OXPHOS impairment in MRC mutant cells
Invernizzi, F., D’Amato, I., Jensen, P. B., Ravaglia, S., Zeviani, M., & Tiranti, V. (2012) · Mitochondrion · doi:10.1016/j.mito.2012.01.001 · Cited by 91 · see citing works
Self-citationCarrozzo, R., Verrigni, D., Rasmussen, M., de Coo, R., Amartino, H., Bianchi, M., Buhas, D., Mesli, S., Naess, K., Born, A. P., et al. (2016) · Journal of Inherited Metabolic Disease · doi:10.1007/s10545-015-9894-9 · Cited by 90 · see citing works
Nouws, J., Nijtmans, L. G. J., Smeitink, J. A. M., & Vogel, R. O. (2011) · Brain · doi:10.1093/brain/awr261 · Cited by 87 · see citing works
Ret rescues mitochondrial morphology and muscle degeneration of Drosophila Pink1 mutants
Klein, P., Müller-Rischart, A. K., Motori, E., Schönbauer, C., Schnorrer, F., Winklhofer, K. F., & Klein, R. (2014) · The EMBO Journal · doi:10.1002/embj.201284290 · Cited by 87 · see citing works
Baruffini, E., Dallabona, C., Invernizzi, F., Yarham, J. W., Melchionda, L., Blakely, E. L., Lamantea, E., Donnini, C., Santra, S., Vijayaraghavan, S., et al. (2013) · Human Mutation · doi:10.1002/humu.22393 · Cited by 86 · see citing works
Self-citationThe p.M292T NDUFS2 mutation causes complex I-deficient Leigh syndrome in multiple families
Tuppen, H. A. L., Hogan, V. E., He, L., Blakely, E. L., Worgan, L. C., Aldosary, M. S., Saretzki, G. C., Alston, C. L., Morris, A. A. M., Clarke, M. A., et al. (2010) · Brain · doi:10.1093/brain/awq232 · Cited by 82 · see citing works
Effects of riboflavin in children with complex II deficiency
Bugiani, M., Lamantea, E., Invernizzi, F., Moroni, I., Bizzi, A., Zeviani, M., & Uziel, G. (2006) · Brain and Development · doi:10.1016/j.braindev.2006.04.001 · Cited by 81 · see citing works
Self-citationPapa, S., Rasmo, D. D., Technikova-Dobrova, Z., Panelli, D., Signorile, A., Scacco, S., Petruzzella, V., Papa, F., Palmisano, G., Gnoni, A., et al. (2011) · FEBS Letters · doi:10.1016/j.febslet.2011.09.019 · Cited by 75 · see citing works
Sofou, K., de Coo, I. F. M., Østergaard, E., Isohanni, P., Naess, K., Meırleır, L. D., Tzoulis, C., Uusimaa, J., Lönnqvist, T. R. I., Bindoff, L. A., et al. (2017) · Journal of Medical Genetics · doi:10.1136/jmedgenet-2017-104891 · Cited by 75 · see citing works
Friederich, M. W., Erdogan, A. J., Coughlin, C. R., Elos, M. T., Jiang, H. L., O’Rourke, C. P., Lovell, M. A., Wartchow, E. P., Gowan, K., Chatfield, K. C., et al. (2016) · Human Molecular Genetics · doi:10.1093/hmg/ddw431 · Cited by 74 · see citing works
Mitochondrial dysfunction in central nervous system white matter disorders
Morató, L., Bertini, E., Verrigni, D., Ardissone, A., Ruíz, M., Ferrer, I., Uziel, G., & Pujol, A. (2014) · Glia · doi:10.1002/glia.22670 · Cited by 75 · see citing works
Self-citationKamiński, M. M., Röth, D., Krammer, P. H., & Gülow, K. (2013) · Archivum Immunologiae et Therapiae Experimentalis · doi:10.1007/s00005-013-0235-0 · Cited by 72 · see citing works
Hepatocerebral Form of Mitochondrial DNA Depletion Syndrome
Spinazzola, A., Santer, R., Akman, O. H., Tsiakas, K., Schaefer, H., Ding, X. Q., Karadimas, C. L., Shanske, S., Ganesh, J., Mauro, S. D., et al. (2008) · Archives of Neurology · doi:10.1001/archneur.65.8.1108 · Cited by 73 · see citing works
Self-citationMelchionda, L., Haack, T. B., Hardy, S. A., Abbink, T. E. M., Fernández‐Vizarra, E., Lamantea, E., Marchet, S., Morandi, L. O., Moggio, M. G., Carrozzo, R., et al. (2014) · The American Journal of Human Genetics · doi:10.1016/j.ajhg.2014.08.003 · Cited by 73 · see citing works
Self-citationInvernizzi, F., Tigano, M., Dallabona, C., Donnini, C., Ferrero, I., Cremonte, M., Ghezzi, D., Lamperti, C., & Zeviani, M. (2013) · Human Mutation · doi:10.1002/humu.22441 · Cited by 72 · see citing works
Self-citationNAD+ repletion with niacin counteracts cancer cachexia
Beltrà, M., Pöllänen, N., Fornelli, C., Tonttila, K., Hsu, M. Y., Zampieri, S., Moletta, L., Corrà, S., Porporato, P. E., Kivelä, R., et al. (2023) · Nature Communications · doi:10.1038/s41467-023-37595-6 · Cited by 71 · see citing works
Taddei, M. L., Giannoni, E., Raugei, G., Scacco, S., Sardanelli, A. M., Papa, S., & Chiarugi, P. (2012) · Journal of Signal Transduction · doi:10.1155/2012/684592 · Cited by 70 · see citing works
Defective mitochondrial rRNA methyltransferase MRM2 causes MELAS-like clinical syndrome
Garone, C., D’Souza, A. R., Dallabona, C., Lodi, T., Rebelo‐Guiomar, P., Rorbach, J., Donati, M. A., Procopio, E., Montomoli, M., Guerrini, R., et al. (2017) · Human Molecular Genetics · doi:10.1093/hmg/ddx314 · Cited by 70 · see citing works
Self-citationBiallelic Mutations in TMEM126B Cause Severe Complex I Deficiency with a Variable Clinical Phenotype
Alston, C. L., Compton, A. G., Formosa, L. E., Strecker, V., Oláhová, M., Haack, T. B., Smet, J., Stouffs, K., Diakumis, P., Ciara, E., et al. (2016) · The American Journal of Human Genetics · doi:10.1016/j.ajhg.2016.05.021 · Cited by 67 · see citing works
Iommarini, L., Calvaruso, M. A., Kurelac, I., Gasparre, G., & Porcelli, A. M. (2012) · The International Journal of Biochemistry & Cell Biology · doi:10.1016/j.biocel.2012.05.016 · Cited by 66 · see citing works
Mitochondrial complex I deficiency of nuclear origin
Pagniez-Mammeri, H., Loublier, S., Legrand, A., Bénit, P., Rustin, P., & Slama, A. (2011) · Molecular Genetics and Metabolism · doi:10.1016/j.ymgme.2011.11.188 · Cited by 64 · see citing works
A novel AIFM1 mutation expands the phenotype to an infantile motor neuron disease
Diodato, D., Tasca, G. A., Verrigni, D., D’Amico, A., Rizza, T., Tozzi, G., Martinelli, D., Verardo, M., Invernizzi, F., Nasca, A., et al. (2015) · European Journal of Human Genetics · doi:10.1038/ejhg.2015.141 · Cited by 64 · see citing works
Self-citationMeo, I. D., Marchet, S., Lamperti, C., Zeviani, M., & Viscomi, C. F. (2017) · Gene Therapy · doi:10.1038/gt.2017.53 · Cited by 62 · see citing works
Self-citationGranatiero, V., Giorgio, V., Calí, T., Patrón, M., Brini, M., Bernardi, P., Tiranti, V., Zeviani, M., Pallafacchina, G., Stefani, D. D., et al. (2015) · Cell Death and Differentiation · doi:10.1038/cdd.2015.84 · Cited by 62 · see citing works
Self-citationSpinazzi, M., Casarin, A., Pertegato, V., Ermani, M., Salviati, L., & Angelini, C. I. (2011) · Mitochondrion · doi:10.1016/j.mito.2011.07.006 · Cited by 61 · see citing works
Carrozzo, R., Torraco, A., Fiermonte, G., Martinelli, D., Nottia, M. D., Rizza, T., Vozza, A., Verrigni, D., Diodato, D., Parisi, G., et al. (2014) · Mitochondrion · doi:10.1016/j.mito.2014.09.006 · Cited by 61 · see citing works
Ruiter, E. M., Siers, M. H., van den Elzen, C., van Engelen, B. G. M., Smeitink, J. A. M., Rodenburg, R. J., & Hol, F. A. (2006) · European Journal of Human Genetics · doi:10.1038/sj.ejhg.5201735 · Cited by 61 · see citing works
Cortopassi, G. A., Danielson, S. R., Alemi, M., Zhan, S. S., Tong, W., Carelli, V., Martinuzzi, A., Marzuki, S., Majamaa, K., & Wong, A. (2006) · Mitochondrion · doi:10.1016/j.mito.2006.05.002 · Cited by 60 · see citing works
Wang, S., Weng, W., Lee, N., Hwu, W., Fan, P., & Lee, W. (2008) · Pediatrics & Neonatology · doi:10.1016/s1875-9572(08)60030-3 · Cited by 60 · see citing works
Bottani, E., Giordano, C., Civiletto, G., Meo, I. D., Auricchio, A., Ciusani, E., Marchet, S., Lamperti, C., d’Amati, G., Viscomi, C. F., et al. (2013) · Molecular Therapy · doi:10.1038/mt.2013.230 · Cited by 58 · see citing works
Self-citationCavitating leukoencephalopathy with multiple mitochondrial dysfunction syndrome and NFU1 mutations
Invernizzi, F., Ardissone, A., Lamantea, E., Garavaglia, B., Zeviani, M., Farina, L., Ghezzi, D., & Moroni, I. (2014) · Frontiers in Genetics · doi:10.3389/fgene.2014.00412 · Cited by 58 · see citing works
Self-citationA novel mutation in the human complex I NDUFS7 subunit associated with Leigh syndrome
Lebon, S., Rodriguez, D., Bridoux, D., Zerrad-Saadi, A., Rötig, A., Münnich, A., Legrand, A., & Slama, A. (2007) · Molecular Genetics and Metabolism · doi:10.1016/j.ymgme.2006.12.007 · Cited by 57 · see citing works
Early-Onset Ophthalmoplegia in Leigh-Like Syndrome Due to NDUFV1 Mutations
Laugel, V., This-Bernd, V., Cormier‐Daire, V., Speeg‐Schatz, C., Martin, A. D. S., & Fischbach, M. (2006) · Pediatric Neurology · doi:10.1016/j.pediatrneurol.2006.08.007 · Cited by 57 · see citing works
Fernández‐Vizarra, E., Berardinelli, A. L., Valente, L., Tiranti, V., & Zeviani, M. (2009) · BMJ Case Reports · doi:10.1136/bcr.05.2009.1889 · Cited by 57 · see citing works
Self-citationRiboflavin metabolism: role in mitochondrial function
Balasubramaniam, S., & Yaplito‐Lee, J. (2020) · Journal of Translational Genetics and Genomics · doi:10.20517/jtgg.2020.34 · Cited by 55 · see citing works
Mitochondrial Neurodegeneration
Zeviani, M., & Viscomi, C. F. (2022) · Cells · doi:10.3390/cells11040637 · Cited by 54 · see citing works
Self-citationThe unique neuroradiology of complex I deficiency due to NDUFA12L defect
Barghuti, F., Elian, K., Gomori, J. M., Shaag, A., Edvardson, S., Saada, A., & Elpeleg, O. N. (2008) · Molecular Genetics and Metabolism · doi:10.1016/j.ymgme.2007.11.013 · Cited by 52 · see citing works
Tonin, Y., Heckel, A., Vysokikh, M. Y., Dovydenko, I. S., Meschaninova, M. I., Rötig, A., Münnich, A., Venyaminova, A. G., Tarassov, I., & Entelis, N. (2014) · Journal of Biological Chemistry · doi:10.1074/jbc.m113.528968 · Cited by 52 · see citing works
Glucose metabolism and diet-based prevention of liver dysfunction in MPV17 mutant patients
Parini, R., Furlan, F., Notarangelo, L. D., Spinazzola, A., Uziel, G., Strisciuglio, P., Concolino, D., Corbetta, C., Nebbia, G., Menni, F., et al. (2008) · Journal of Hepatology · doi:10.1016/j.jhep.2008.08.019 · Cited by 52 · see citing works
Self-citationNovel TTC19 mutation in a family with severe psychiatric manifestations and complex III deficiency
Nogueira, C., Barros, J., Sá, M. J., Azevedo, L., Taipa, R. J. F., Torraco, A., Meschini, M. C., Verrigni, D., Nesti, C., Rizza, T., et al. (2013) · Neurogenetics · doi:10.1007/s10048-013-0361-1 · Cited by 51 · see citing works
Verrigni, D., Nottia, M. D., Ardissone, A., Baruffini, E., Nasca, A., Legati, A., Bellacchio, E., Fagiolari, G., Martinelli, D., Fusco, L., et al. (2019) · Human Mutation · doi:10.1002/humu.23729 · Cited by 51 · see citing works
Self-citationVarghese, F., Atcheson, E., Bridges, H. R., & Hirst, J. (2015) · Human Molecular Genetics · doi:10.1093/hmg/ddv344 · Cited by 50 · see citing works
Effects of levosimendan on mitochondrial function in patients with septic shock: A randomized trial
Torraco, A., Carrozzo, R., Piemonte, F., Pastore, A., Tozzi, G., Verrigni, D., Assenza, M., Orecchioni, A., D’Egidio, A., Maraffa, E., et al. (2014) · Biochimie · doi:10.1016/j.biochi.2014.03.006 · Cited by 50 · see citing works
Novel mutations in IBA57 are associated with leukodystrophy and variable clinical phenotypes
Torraco, A., Ardissone, A., Invernizzi, F., Rizza, T., Fiermonte, G., Niceta, M., Zanetti, N., Martinelli, D., Vozza, A., Verrigni, D., et al. (2016) · Journal of Neurology · doi:10.1007/s00415-016-8312-z · Cited by 50 · see citing works
Self-citationTorraco, A., Peralta, S., Iommarini, L., & Díaz, F. (2015) · Mitochondrion · doi:10.1016/j.mito.2015.01.009 · Cited by 50 · see citing works
Metabolic flexibility of mitochondrial respiratory chain disorders predicted by computer modelling
Zielinski, L. P., Smith, A. C., Smith, A., & Robinson, A. J. (2016) · Mitochondrion · doi:10.1016/j.mito.2016.09.003 · Cited by 49 · see citing works
Bandara, A. B. P. A., Drake, J. C., James, C. C., Smyth, J. W., & Brown, D. A. (2021) · Mitochondrion · doi:10.1016/j.mito.2021.03.003 · Cited by 48 · see citing works
Chouchani, E. T., Methner, C., Buonincontri, G., Hu, C.-H., Logan, A., Sawiak, S. J., Murphy, M. P., & Krieg, T. (2014) · PLoS ONE · doi:10.1371/journal.pone.0094157 · Cited by 48 · see citing works
Ferreira, M., Torraco, A., Rizza, T., Fattori, F., Meschini, M. C., Castana, C., Go, N. E., Nargang, F. E., Duarte, M., Piemonte, F., et al. (2011) · Neurogenetics · doi:10.1007/s10048-010-0265-2 · Cited by 48 · see citing works
Mitochondrial DNA G8363A mutation in the tRNALys gene: Clinical, biochemical and pathological study
Virgilio, R., Ronchi, D., Bordoni, A., Fassone, E., Bonato, S., Donadoni, C., Torgano, G., Moggio, M. G., Corti, S. P., Bresolin, N., et al. (2009) · Journal of the Neurological Sciences · doi:10.1016/j.jns.2009.01.025 · Cited by 47 · see citing works
- Authors
- Bugiani, M., Invernizzi, F., Alberio, S., Briem, E., Lamantea, E., Carrara, F., Moroni, I., Farina, L., Spada, M., Donati, M. A., Uziel, G., & Zeviani, M.
- Affiliations
- Fondazione IRCCS Istituto Neurologico Carlo Besta (IT)
- Ospedale Regina Margherita (IT)
- Meyer Children's Hospital (IT)
- Corresponding author
- Zeviani, M.
- Venue
- Biochimica et Biophysica Acta (BBA) - Bioenergetics
- Publisher
- Elsevier BV
- Published
- 2004-12
- Volume
- 1659
- Issue
- 2-3
- Pages
- 136-147
- ISSN
- 0005-2728 (ISSN-L)
- OpenAlex
- W2077446001
- Cited by
- 264
- References
- 44
- Citations by year
- 2026: 5 · 2025: 6 · 2024: 10 · 2023: 7 · 2022: 8 · 2021: 13 · 2020: 15 · 2019: 12 · 2018: 14 · 2017: 15 · 2016: 16 · 2015: 15 · 2014: 15 · 2013: 17 · 2012: 17
- FWCI
- 2.72
- Type
- journal article
- Language
- en
- Open access
- bronze
- doi.org
- Licence
- Locations
- Biochimica et Biophysica Acta (BBA) - Bioenergetics (publishedVersion)
- PubMed (publishedVersion)
- Institutional Research Information System University of Turin (University of Turin) (submittedVersion)
- Padua Research Archive (University of Padova) (submittedVersion)
- Topics
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Mitochondrial Function and Pathology
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Clinical Biochemistry › Metabolism and Genetic Disorders
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Genetics › Genomics and Rare Diseases
- Keywords
- complex I deficiency, mitochondrial disease, Leigh syndrome, neonatal cardiomyopathy, mitochondrial encephalomyopathy, NDUFS1, NDUFS2, NDUFS7, mitochondrial DNA mutations, genetic heterogeneity, children
- MeSH terms
- Acidosis, Lactic, Child, DNA, Mitochondrial, Humans, Infant, Iron-Sulfur Proteins, Leigh Disease, Leukoencephalopathy, Progressive Multifocal, Metabolism, Inborn Errors, Mutation, Cardiomyopathies, NADH Dehydrogenase, Proteins, NAD(P)H Dehydrogenase (Quinone), Mitochondrial Proteins, Electron Transport Complex I
About these checks
Registry, retraction, Cites and statistics results are computed, not judged, with one exception: a printed reference that no index lists is read once by a model before it counts as a citation error. Claims are extracted by a model and kept only when their quote appears verbatim in the text; each cited source is then checked, with a model’s help, for whether it says what the claim relies on. Those verdicts can be wrong — read the claim’s quote and the cited source before relying on one.
Statistics and arithmetic are recomputed, not judged.
Confidence bands are shown only for fields calibrated on our annotated test set. None is calibrated yet, so no band is shown.
What counts as a claim ›How we measure confidence ›How we check this ›
Are you an author, or do you see a problem with this page? Request a review
