Publications

Found 217 results
Filters: Keyword is Mitochondrial Proteins  [Clear All Filters]
2009
Poulton J & Holt IJ (2009) 163rd ENMC International Workshop: nucleoid and nucleotide biology in syndromes of mitochondrial DNA depletion myopathy 12-14 December 2008, Naarden, The Netherlands. Neuromuscul Disord 19, 439-43
Dimmock D, Maranda B, Dionisi-Vici C, Wang J, Kleppe S, Fiermonte G, Bai R, Hainline B, Hamosh A, O'Brien WE, Scaglia F & Wong L-J (2009) Citrin deficiency, a perplexing global disorder. Mol Genet Metab 96, 44-9
Mao C-C & Holt IJ (2009) Clinical and molecular aspects of diseases of mitochondrial DNA instability. Chang Gung Med J 32, 354-69
Iacovino M, Granycome C, Sembongi H, Bokori-Brown M, Butow RA, Holt IJ & Bateman JM (2009) The conserved translocase Tim17 prevents mitochondrial DNA loss. Hum Mol Genet 18, 65-74
Parker N, Vidal-Puig AJ, Azzu V & Brand MD (2009) Dysregulation of glucose homeostasis in nicotinamide nucleotide transhydrogenase knockout mice is independent of uncoupling protein 2. Biochim Biophys Acta 1787, 1451-7
Viscomi C, Spinazzola A, Maggioni M, Fernandez-Vizarra E, Massa V, Pagano C, Vettor R, Mora M & Zeviani M (2009) Early-onset liver mtDNA depletion and late-onset proteinuric nephropathy in Mpv17 knockout mice. Hum Mol Genet 18, 12-26
Lamperti C & Zeviani M (2009) Encephalomyopathies caused by abnormal nuclear-mitochondrial intergenomic cross-talk. Acta Myol 28, 2-11
Humphrey DM, Toivonen JM, Giannakou M, Partridge L & Brand MD (2009) Expression of human uncoupling protein-3 in Drosophila insulin-producing cells increases insulin-like peptide (DILP) levels and shortens lifespan. Exp Gerontol 44, 316-27
Fernandez-Ayala DJM, Sanz A, Vartiainen S, Kemppainen KK, Babusiak M, Mustalahti E, Costa R, Tuomela T, Zeviani M, Chung J, O'Dell KMC, Rustin P & Jacobs HT (2009) Expression of the Ciona intestinalis alternative oxidase (AOX) in Drosophila complements defects in mitochondrial oxidative phosphorylation. Cell Metab 9, 449-60
Parini R, Furlan F, Notarangelo L, Spinazzola A, Uziel G, Strisciuglio P, Concolino D, Corbetta C, Nebbia G, Menni F, Rossi G, Maggioni M & Zeviani M (2009) Glucose metabolism and diet-based prevention of liver dysfunction in MPV17 mutant patients. J Hepatol 50, 215-21
Malena A, Loro E, Di Re M, Holt IJ & Vergani L (2009) Inhibition of mitochondrial fission favours mutant over wild-type mitochondrial DNA. Hum Mol Genet 18, 3407-16
Singh A, Wirtz M, Parker N, Hogan M, Strahler J, Michailidis G, Schmidt S, Vidal-Puig A, Diano S, Andrews P, Brand MD & Friedman J (2009) Leptin-mediated changes in hepatic mitochondrial metabolism, structure, and protein levels. Proc Natl Acad Sci U S A 106, 13100-5
Tiranti V, Viscomi C, Hildebrandt T, Di Meo I, Mineri R, Tiveron C, Levitt MD, Prelle A, Fagiolari G, Rimoldi M & Zeviani M (2009) Loss of ETHE1, a mitochondrial dioxygenase, causes fatal sulfide toxicity in ethylmalonic encephalopathy. Nat Med 15, 200-5
Affourtit C & Brand MD (2009) Measuring mitochondrial bioenergetics in INS-1E insulinoma cells. Methods Enzymol 457, 405-24
Crichton PG, Parker N, Vidal-Puig AJ & Brand MD (2009) Not all mitochondrial carrier proteins support permeability transition pore formation: no involvement of uncoupling protein 1. Biosci Rep 30, 187-92
Parker N, Crichton PG, Vidal-Puig AJ & Brand MD (2009) Uncoupling protein-1 (UCP1) contributes to the basal proton conductance of brown adipose tissue mitochondria. J Bioenerg Biomembr 41, 335-42
2008
Azzu V, Affourtit C, Breen EP, Parker N & Brand MD (2008) Dynamic regulation of uncoupling protein 2 content in INS-1E insulinoma cells. Biochim Biophys Acta 1777, 1378-83
Parker N, Affourtit C, Vidal-Puig A & Brand MD (2008) Energization-dependent endogenous activation of proton conductance in skeletal muscle mitochondria. Biochem J 412, 131-9
Wiedmer A, Wang P, Zhou J, Rennekamp AJ, Tiranti V, Zeviani M & Lieberman PM (2008) Epstein-Barr virus immediate-early protein Zta co-opts mitochondrial single-stranded DNA binding protein to promote viral and inhibit mitochondrial DNA replication. J Virol 82, 4647-55
Spinazzola A, Santer R, Akman OH, Tsiakas K, Schaefer H, Ding X, Karadimas CL, Shanske S, Ganesh J, Di Mauro S & Zeviani M (2008) Hepatocerebral form of mitochondrial DNA depletion syndrome: novel MPV17 mutations. Arch Neurol 65, 1108-13
Mineri R, Rimoldi M, Burlina AB, Koskull S, Perletti C, Heese B, von Döbeln U, Mereghetti P, Di Meo I, Invernizzi F, Zeviani M, Uziel G & Tiranti V (2008) Identification of new mutations in the ETHE1 gene in a cohort of 14 patients presenting with ethylmalonic encephalopathy. J Med Genet 45, 473-8
Whitworth AJ, Lee JR, Ho VM-W, Flick R, Chowdhury R & G McQuibban A (2008) Rhomboid-7 and HtrA2/Omi act in a common pathway with the Parkinson's disease factors Pink1 and Parkin. Dis Model Mech 1, 168-74; discussion 173
Affourtit C & Brand MD (2008) On the role of uncoupling protein-2 in pancreatic beta cells. Biochim Biophys Acta 1777, 973-9
Parker N, Vidal-Puig A & Brand MD (2008) Stimulation of mitochondrial proton conductance by hydroxynonenal requires a high membrane potential. Biosci Rep 28, 83-8
Cízková A, Stránecký V, Mayr JA, Tesarova M, Havlícková V, Paul J, Ivánek R, Kuss AW, Hansikova H, Kaplanová V, Vrbacký M, Hartmannová H, Nosková L, Honzík T, Drahota Z, Magner M, Hejzlarová K, Sperl W, Zeman J, Houštěk J & Kmoch S (2008) TMEM70 mutations cause isolated ATP synthase deficiency and neonatal mitochondrial encephalocardiomyopathy. Nat Genet 40, 1288-90

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