Found 437 results
Filters: Keyword is Mitochondria  [Clear All Filters]
Rovio A, Tiranti V, Bednarz AL, Suomalainen A, Spelbrink JN, Lecrenier N, Melberg A, Zeviani M, Poulton J, Foury F & Jacobs HT (1999) Analysis of the trinucleotide CAG repeat from the human mitochondrial DNA polymerase gene in healthy and diseased individuals. Eur J Hum Genet 7, 140-6
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
Stock D, Leslie AG & Walker JE (1999) Molecular architecture of the rotary motor in ATP synthase. Science 286, 1700-5
Dionisi-Vici C, Seneca S, Zeviani M, Fariello G, Rimoldi M, Bertini E & De Meirleir L (1998) Fulminant Leigh syndrome and sudden unexpected death in a family with the T9176C mutation of the mitochondrial ATPase 6 gene. J Inherit Metab Dis 21, 2-8
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
Casari G, De Fusco M, Ciarmatori S, Zeviani M, Mora M, Fernandez P, De Michele G, Filla A, Cocozza S, Marconi R, Dürr A, Fontaine B & Ballabio A (1998) Spastic paraplegia and OXPHOS impairment caused by mutations in paraplegin, a nuclear-encoded mitochondrial metalloprotease. Cell 93, 973-83
Arnold I, Pfeiffer K, Neupert W, Stuart RA & Schägger H (1998) Yeast mitochondrial F1F0-ATP synthase exists as a dimer: identification of three dimer-specific subunits. EMBO J 17, 7170-8
Dziembowski A, Malewicz M, Minczuk M, Golik P, Dmochowska A & Stepien PP (1998) The yeast nuclear gene DSS1, which codes for a putative RNase II, is necessary for the function of the mitochondrial degradosome in processing and turnover of RNA. Mol Gen Genet 260, 108-14
Uhlin U, Cox GB & Guss JM (1997) Crystal structure of the epsilon subunit of the proton-translocating ATP synthase from Escherichia coli. Structure 5, 1219-30
Zeviani M, Petruzzella V & Carrozzo R (1997) Disorders of nuclear-mitochondrial intergenomic signalling. J Bioenerg Biomembr 29, 121-30
Tiranti V, Savoia A, Forti F, D'Apolito MF, Centra M, Rocchi M & Zeviani M (1997) Identification of the gene encoding the human mitochondrial RNA polymerase (h-mtRPOL) by cyberscreening of the Expressed Sequence Tags database. Hum Mol Genet 6, 615-25
Dunbar DR, Moonie PA, Zeviani M & Holt IJ (1996) Complex I deficiency is associated with 3243G:C mitochondrial DNA in osteosarcoma cell cybrids. Hum Mol Genet 5, 123-29
Tiranti V, Rossi E, Ruiz-Carrillo A, Rossi G, Rocchi M, DiDonato S, Zuffardi O & Zeviani M (1995) Chromosomal localization of mitochondrial transcription factor A (TCF6), single-stranded DNA-binding protein (SSBP), and endonuclease G (ENDOG), three human housekeeping genes involved in mitochondrial biogenesis. Genomics 25, 559-64
Antozzi C, Franceschetti S, Filippini G, Barbiroli B, Savoiardo M, Fiacchino F, Rimoldi M, Lodi R, Zaniol P & Zeviani M (1995) Epilepsia partialis continua associated with NADH-coenzyme Q reductase deficiency. J Neurol Sci 129, 152-61
Tiranti V, Rossi E, Rocchi M, DiDonato S, Zuffardi O & Zeviani M (1995) The gene (NFE2L1) for human NRF-1, an activator involved in nuclear-mitochondrial interactions, maps to 7q32. Genomics 27, 555-7
Zoratti M & Szabò I (1995) The mitochondrial permeability transition. Biochim Biophys Acta 1241, 139-76
Tiranti V, Munaro M, Sandonà D, Lamantea E, Rimoldi M, DiDonato S, Bisson R & Zeviani M (1995) Nuclear DNA origin of cytochrome c oxidase deficiency in Leigh's syndrome: genetic evidence based on patient's-derived rho degrees transformants. Hum Mol Genet 4, 2017-23
Mariotti C, Tiranti V, Carrara F, Dallapiccola B, DiDonato S & Zeviani M (1994) Defective respiratory capacity and mitochondrial protein synthesis in transformant cybrids harboring the tRNA(Leu(UUR)) mutation associated with maternally inherited myopathy and cardiomyopathy. J Clin Invest 93, 1102-7
Zeviani M & Taroni F (1994) Mitochondrial diseases. Baillieres Clin Neurol 3, 315-34
Walker JE (1994) The regulation of catalysis in ATP synthase. Curr Opin Struct Biol 4, 912-8
Yan WL, Lerner TJ, Haines JL & Gusella JF (1994) Sequence analysis and mapping of a novel human mitochondrial ATP synthase subunit 9 cDNA (ATP5G3). Genomics 24, 375-7
Curth U, Urbanke C, Greipel J, Gerberding H, Tiranti V & Zeviani M (1994) Single-stranded-DNA-binding proteins from human mitochondria and Escherichia coli have analogous physicochemical properties. Eur J Biochem 221, 435-43
Tiranti V, Barat-Gueride B, Bijl J, DiDonato S & Zeviani M (1991) A full-length cDNA encoding a mitochondrial DNA-specific single-stranded DNA binding protein from Xenopus laevis. Nucleic Acids Res 19, 4291