Found 27 results
Filters: Keyword is Electron Transport Complex III  [Clear All Filters]
Manole A, Jaunmuktane Z, Hargreaves I, Ludtmann MHR, Salpietro V, Bello OD, Pope S, Pandraud A, Horga A, Scalco RS, Li A, Ashokkumar B, Lourenço CM, Heales S, Horvath R, Chinnery PF, Toro C, Singleton AB, Jacques TS, Abramov AY, Muntoni F, Hanna MG, Reilly MM, Revesz T, Kullmann DM, Jepson JEC & Houlden H (2017) Clinical, pathological and functional characterization of riboflavin-responsive neuropathy. Brain 140, 2820-2837
Letts JA, Fiedorczuk K & Sazanov LA (2016) The architecture of respiratory supercomplexes. Nature 537, 644-648
Zieliński ŁP, Smith AC, Smith AG & Robinson AJ (2016) Metabolic flexibility of mitochondrial respiratory chain disorders predicted by computer modelling. Mitochondrion 31, 45-55
Invernizzi F, Tigano M, Dallabona C, Donnini C, Ferrero I, Cremonte M, Ghezzi D, Lamperti C & Zeviani M (2013) A homozygous mutation in LYRM7/MZM1L associated with early onset encephalopathy, lactic acidosis, and severe reduction of mitochondrial complex III activity. Hum Mutat 34, 1619-22
Sánchez E, Lobo T, Fox JL, Zeviani M, Winge DR & Fernandez-Vizarra E (2013) LYRM7/MZM1L is a UQCRFS1 chaperone involved in the last steps of mitochondrial Complex III assembly in human cells. Biochim Biophys Acta 1827, 285-93
Ghezzi D, Arzuffi P, Zordan M, Da Re C, Lamperti C, Benna C, D'Adamo P, Diodato D, Costa R, Mariotti C, Uziel G, Smiderle C & Zeviani M (2011) Mutations in TTC19 cause mitochondrial complex III deficiency and neurological impairment in humans and flies. Nat Genet 43, 259-63
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
Mineri R, Pavelka N, Fernandez-Vizarra E, Ricciardi-Castagnoli P, Zeviani M & Tiranti V (2009) How do human cells react to the absence of mitochondrial DNA?. PLoS One 4, e5713
Fernandez-Vizarra E, Bugiani M, Goffrini P, Carrara F, Farina L, Procopio E, Donati A, Uziel G, Ferrero I & Zeviani M (2007) Impaired complex III assembly associated with BCS1L gene mutations in isolated mitochondrial encephalopathy. Hum Mol Genet 16, 1241-52
Cortelli P, Mandrioli J, Zeviani M, Lodi R, Prata C, Pecorari M, Orlando G & Guaraldi G (2007) Mitochondrial complex III deficiency in a case of HCV related noninflammatory myopathy. J Neurol 254, 1450-2
Bell EL, Klimova TA, Eisenbart J, Moraes CT, Murphy MP, Budinger GRScott & Chandel NS (2007) The Qo site of the mitochondrial complex III is required for the transduction of hypoxic signaling via reactive oxygen species production. J Cell Biol 177, 1029-36
Helmerhorst EJ, Stan M, Murphy MP, Sherman F & Oppenheim FG (2005) The concomitant expression and availability of conventional and alternative, cyanide-insensitive, respiratory pathways in Candida albicans. Mitochondrion 5, 200-11
Iwaki M, Yakovlev G, Hirst J, Osyczka A, P Dutton L, Marshall D & Rich PR (2005) Direct observation of redox-linked histidine protonation changes in the iron-sulfur protein of the cytochrome bc1 complex by ATR-FTIR spectroscopy. Biochemistry 44, 4230-7
Brunelle JK, Bell EL, Quesada NM, Vercauteren K, Tiranti V, Zeviani M, Scarpulla RC & Chandel NS (2005) Oxygen sensing requires mitochondrial ROS but not oxidative phosphorylation. Cell Metab 1, 409-14
Leggate EJ & Hirst J (2005) Roles of the disulfide bond and adjacent residues in determining the reduction potentials and stabilities of respiratory-type Rieske clusters. Biochemistry 44, 7048-58
Miwa S & Brand MD (2005) The topology of superoxide production by complex III and glycerol 3-phosphate dehydrogenase in Drosophila mitochondria. Biochim Biophys Acta 1709, 214-9
de Oliveira FTiago, Bominaar EL, Hirst J, Fee JA & Münck E (2004) Antisymmetric exchange in [2Fe-2S]1+ clusters: EPR of the Rieske protein from Thermus thermophilus at pH 14. J Am Chem Soc 126, 5338-9
Leggate EJ, Bill E, Essigke T, G Ullmann M & Hirst J (2004) Formation and characterization of an all-ferrous Rieske cluster and stabilization of the [2Fe-2S]0 core by protonation. Proc Natl Acad Sci U S A 101, 10913-8
Hunsicker-Wang LM, Heine A, Chen Y, Luna EP, Todaro T, Zhang YMing, Williams PA, McRee DE, Hirst J, C Stout D & Fee JA (2003) High-resolution structure of the soluble, respiratory-type Rieske protein from Thermus thermophilus: analysis and comparison. Biochemistry 42, 7303-17
Zeviani M & Spinazzola A (2003) Mitochondrial disorders. Curr Neurol Neurosci Rep 3, 423-32
Zu Y, Couture MM-J, Kolling DRJ, Crofts AR, Eltis LD, Fee JA & Hirst J (2003) Reduction potentials of Rieske clusters: importance of the coupling between oxidation state and histidine protonation state. Biochemistry 42, 12400-8
Miwa S, St-Pierre J, Partridge L & Brand MD (2003) Superoxide and hydrogen peroxide production by Drosophila mitochondria. Free Radic Biol Med 35, 938-48