Found 99 results
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Yu-Wai-Man P, Brown DT, Wehnert MS, Zeviani M, Carrara F, Turnbull DM & Chinnery PF (2002) NDUFA-1 is not a nuclear modifier gene in Leber hereditary optic neuropathy. Neurology 58, 1861-2
Bruno C, Biancheri R, Garavaglia B, Biedi C, Rossi A, Lamba LDoria, Bado M, Greco M, Zeviani M & Minetti C (2002) A novel mutation in the SURF1 gene in a child with Leigh disease, peripheral neuropathy, and cytochrome-c oxidase deficiency. J Child Neurol 17, 233-6
Jovine L, Park J & Wassarman PM (2002) Sequence similarity between stereocilin and otoancorin points to a unified mechanism for mechanotransduction in the mammalian inner ear. BMC Cell Biol 3, 28
Echtay KS, Roussel D, St-Pierre J, Jekabsons MB, Cadenas S, Stuart JA, Harper JA, Roebuck SJ, Morrison A, Pickering S, Clapham JC & Brand MD (2002) Superoxide activates mitochondrial uncoupling proteins. Nature 415, 96-9
Päivärinne H & Kainulainen H (2001) DAPIT, a novel protein down-regulated in insulin-sensitive tissues in streptozotocin-induced diabetes. Acta Diabetol 38, 83-6
Murphy MP, Leuenberger D, Curran SP, Oppliger W & Koehler CM (2001) The essential function of the small Tim proteins in the TIM22 import pathway does not depend on formation of the soluble 70-kilodalton complex. Mol Cell Biol 21, 6132-8
Stuart JA, Cadenas S, Jekabsons MB, Roussel D & Brand MD (2001) Mitochondrial proton leak and the uncoupling protein 1 homologues. Biochim Biophys Acta 1504, 144-58
Stuart JA, Harper JA, Brindle KM, Jekabsons MB & Brand MD (2001) A mitochondrial uncoupling artifact can be caused by expression of uncoupling protein 1 in yeast. Biochem J 356, 779-89
Harper JA, Dickinson K & Brand MD (2001) Mitochondrial uncoupling as a target for drug development for the treatment of obesity. Obes Rev 2, 255-65
Arechaga I, Ledesma A & Rial E (2001) The mitochondrial uncoupling protein UCP1: a gated pore. IUBMB Life 52, 165-73
Pequignot MO, Dey R, Zeviani M, Tiranti V, Godinot C, Poyau A, Sue C, Di Mauro S, Abitbol M & Marsac C (2001) Mutations in the SURF1 gene associated with Leigh syndrome and cytochrome C oxidase deficiency. Hum Mutat 17, 374-81
Pequignot MO, Desguerre I, Dey R, Tartari M, Zeviani M, Agostino A, Benelli C, Fouque F, Prip-Buus C, Marchant D, Abitbol M & Marsac C (2001) New splicing-site mutations in the SURF1 gene in Leigh syndrome patients. J Biol Chem 276, 15326-9
Tate CG, Kunji ERS, Lebendiker M & Schuldiner S (2001) The projection structure of EmrE, a proton-linked multidrug transporter from Escherichia coli, at 7 A resolution. EMBO J 20, 77-81
Wittig I, Augstein P, Brown GK, Fujii T, Rotig A, Rustin P, Munnich A, Seibel P, Thorburn D, Wissinger B, Tamboom K, Metspalu A, Lamantea E, Zeviani M & Wehnert MS (2001) Sequence variations in the NDUFA1 gene encoding a subunit of complex I of the respiratory chain. J Inherit Metab Dis 24, 15-27
Nijtmans LG, M Sanz A, Bucko M, Farhoud MH, Feenstra M, Hakkaart GA, Zeviani M & Grivell LA (2001) Shy1p occurs in a high molecular weight complex and is required for efficient assembly of cytochrome c oxidase in yeast. FEBS Lett 498, 46-51
Sazanov LA & Walker JE (2000) Cryo-electron crystallography of two sub-complexes of bovine complex I reveals the relationship between the membrane and peripheral arms. J Mol Biol 302, 455-64
Palmieri L, Lasorsa FM, Vozza A, Agrimi G, Fiermonte G, Runswick MJ, Walker JE & Palmieri F (2000) Identification and functions of new transporters in yeast mitochondria. Biochim Biophys Acta 1459, 363-9
Tiranti V, Galimberti C, Nijtmans L, Bovolenta S, Perini MP & Zeviani M (1999) Characterization of SURF-1 expression and Surf-1p function in normal and disease conditions. Hum Mol Genet 8, 2533-40
Tiranti V, Lamantea E, Uziel G, Zeviani M, Gasparini P, Marzella R, Rocchi M & Fried M (1999) Leigh syndrome transmitted by uniparental disomy of chromosome 9. J Med Genet 36, 927-8
Tiranti V, Jaksch M, Hofmann S, Galimberti C, Hoertnagel K, Lulli L, Freisinger P, Bindoff L, Gerbitz KD, Comi GP, Uziel G, Zeviani M & Meitinger T (1999) Loss-of-function mutations of SURF-1 are specifically associated with Leigh syndrome with cytochrome c oxidase deficiency. Ann Neurol 46, 161-6
Zeviani M, Corona P, Nijtmans L & Tiranti V (1999) Nuclear gene defects in mitochondrial disorders. Ital J Neurol Sci 20, 401-8
Petruzzella V, Tiranti V, Fernandez P, Ianna P, Carrozzo R & Zeviani M (1998) Identification and characterization of human cDNAs specific to BCS1, PET112, SCO1, COX15, and COX11, five genes involved in the formation and function of the mitochondrial respiratory chain. Genomics 54, 494-504
Tiranti V, Hoertnagel K, Carrozzo R, Galimberti C, Munaro M, Granatiero M, Zelante L, Gasparini P, Marzella R, Rocchi M, Bayona-Bafaluy MP, Enriquez JA, Uziel G, Bertini E, Dionisi-Vici C, Franco B, Meitinger T & Zeviani M (1998) Mutations of SURF-1 in Leigh disease associated with cytochrome c oxidase deficiency. Am J Hum Genet 63, 1609-21
Foster DL & Fillingame RH (1982) Stoichiometry of subunits in the H+-ATPase complex of Escherichia coli. J Biol Chem 257, 2009-15