Publications

Found 18 results
Filters: Keyword is Iron-Sulfur Proteins  [Clear All Filters]
2013
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
2009
Ghezzi D, Goffrini P, Uziel G, Horvath R, Klopstock T, Lochmüller H, D'Adamo P, Gasparini P, Strom TM, Prokisch H, Invernizzi F, Ferrero I & Zeviani M (2009) SDHAF1, encoding a LYR complex-II specific assembly factor, is mutated in SDH-defective infantile leukoencephalopathy. Nat Genet 41, 654-6
2007
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
Klinge S, Hirst J, Maman JD, Krude T & Pellegrini L (2007) An iron-sulfur domain of the eukaryotic primase is essential for RNA primer synthesis. Nat Struct Mol Biol 14, 875-7
2006
Bugiani M, Lamantea E, Invernizzi F, Moroni I, Bizzi A, Zeviani M & Uziel G (2006) Effects of riboflavin in children with complex II deficiency. Brain Dev 28, 576-81
2004
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
Bugiani M, Invernizzi F, Alberio S, Briem E, Lamantea E, Carrara F, Moroni I, Farina L, Spada M, Donati MA, Uziel G & Zeviani M (2004) Clinical and molecular findings in children with complex I deficiency. Biochim Biophys Acta 1659, 136-47
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
2003
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
Camba R, Jung Y-S, Hunsicker-Wang LM, Burgess BK, C Stout D, Hirst J & Armstrong FA (2003) Mechanisms of redox-coupled proton transfer in proteins: role of the proximal proline in reactions of the [3Fe-4S] cluster in Azotobacter vinelandii ferredoxin I. Biochemistry 42, 10589-99
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
2002
Zu Y, Fee JA & Hirst J (2002) Breaking and re-forming the disulfide bond at the high-potential, respiratory-type Rieske [2Fe-2S] center of thermus thermophilus: characterization of the sulfhydryl state by protein-film voltammetry. Biochemistry 41, 14054-65
Elliott SJ, Léger C, Pershad HR, Hirst J, Heffron K, Ginet N, Blasco F, Rothery RA, Weiner JH & Armstrong FA (2002) Detection and interpretation of redox potential optima in the catalytic activity of enzymes. Biochim Biophys Acta 1555, 54-9