Publications

Found 28 results
Filters: Keyword is Drosophila melanogaster  [Clear All Filters]
2017
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
2016
Harel T, Yoon WHee, Garone C, Gu S, Coban-Akdemir Z, Eldomery MK, Posey JE, Jhangiani SN, Rosenfeld JA, Cho MT, Fox S, Withers M, Brooks SM, Chiang T, Duraine L, Erdin S, Yuan B, Shao Y, Moussallem E, Lamperti C, Donati MA, Smith JD, McLaughlin HM, Eng CM, Walkiewicz M, Xia F, Pippucci T, Magini P, Seri M, Zeviani M, Hirano M, Hunter JV, Srour M, Zanigni S, Lewis RAlan, Muzny DM, Lotze TE, Boerwinkle E, Gibbs RA, Hickey SE, Graham BH, Yang Y, Buhas D, Martin DM, Potocki L, Graziano C, Bellen HJ & Lupski JR (2016) Recurrent De Novo and Biallelic Variation of ATAD3A, Encoding a Mitochondrial Membrane Protein, Results in Distinct Neurological Syndromes. Am J Hum Genet 99, 831-845
2014
Da-Rè C, Franzolin E, Biscontin A, Piazzesi A, Pacchioni B, Gagliani MCristina, Mazzotta G, Tacchetti C, Zordan MA, Zeviani M, Bernardi P, Bianchi V, De Pittà C & Costa R (2014) Functional characterization of drim2, the Drosophila melanogaster homolog of the yeast mitochondrial deoxynucleotide transporter. J Biol Chem 289, 7448-59
Ivatt RM, Sanchez-Martinez A, Godena VK, Brown S, Ziviani E & Whitworth AJ (2014) Genome-wide RNAi screen identifies the Parkinson disease GWAS risk locus SREBF1 as a regulator of mitophagy. Proc Natl Acad Sci U S A 111, 8494-9
Da-Rè C, von Stockum S, Biscontin A, Millino C, Cisotto P, Zordan MA, Zeviani M, Bernardi P, De Pittà C & Costa R (2014) Leigh syndrome in Drosophila melanogaster: morphological and biochemical characterization of Surf1 post-transcriptional silencing. J Biol Chem 289, 29235-46
Ivatt RM & Whitworth AJ (2014) SREBF1 links lipogenesis to mitophagy and sporadic Parkinson disease. Autophagy 10, 1476-7
Da-Rè C, De Pittà C, Zordan MA, Teza G, Nestola F, Zeviani M, Costa R & Bernardi P (2014) UCP4C mediates uncoupled respiration in larvae of Drosophila melanogaster. EMBO Rep 15, 586-91
2013
Zhang L, Karsten P, Hamm S, Pogson JH, A Muller-Rischart K, Exner N, Haass C, Whitworth AJ, Winklhofer KF, Schulz JB & Voigt A (2013) TRAP1 rescues PINK1 loss-of-function phenotypes. Hum Mol Genet 22, 2829-41
2012
Bricker DK, Taylor EB, Schell JC, Orsak T, Boutron A, Chen Y-C, Cox JE, Cardon CM, Van Vranken JG, Dephoure N, Redin C, Boudina S, Gygi SP, Brivet M, Thummel CS & Rutter J (2012) A mitochondrial pyruvate carrier required for pyruvate uptake in yeast, Drosophila, and humans. Science 337, 96-100
Sanchez-Martinez A, Calleja M, Peralta S, Matsushima Y, Hernandez-Sierra R, Whitworth AJ, Kaguni LS & Garesse R (2012) Modeling pathogenic mutations of human twinkle in Drosophila suggests an apoptosis role in response to mitochondrial defects. PLoS One 7, e43954
2011
Whitworth AJ (2011) Drosophila models of Parkinson's disease. Adv Genet 73, 1-50
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
2010
Ziviani E, Tao RN & Whitworth AJ (2010) Drosophila parkin requires PINK1 for mitochondrial translocation and ubiquitinates mitofusin. Proc Natl Acad Sci U S A 107, 5018-23
Ziviani E & Whitworth AJ (2010) How could Parkin-mediated ubiquitination of mitofusin promote mitophagy?. Autophagy 6, 660-2
2008
Whitworth AJ & Pallanck LJ (2008) Genetic models of Parkinson's disease: mechanisms and therapies. SEB Exp Biol Ser 60, 93-113
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
2006
Whitworth AJ, Wes PD & Pallanck LJ (2006) Drosophila models pioneer a new approach to drug discovery for Parkinson's disease. Drug Discov Today 11, 119-26
Magwere T, Goodall S, Skepper J, Mair W, Brand MD & Partridge L (2006) The effect of dietary restriction on mitochondrial protein density and flight muscle mitochondrial morphology in Drosophila. J Gerontol A Biol Sci Med Sci 61, 36-47
Magwere T, West M, Riyahi K, Murphy MP, Smith RAJ & Partridge L (2006) The effects of exogenous antioxidants on lifespan and oxidative stress resistance in Drosophila melanogaster. Mech Ageing Dev 127, 356-70
Magwere T, Pamplona R, Miwa S, Martinez-Diaz P, Portero-Otín M, Brand MD & Partridge L (2006) Flight activity, mortality rates, and lipoxidative damage in Drosophila. J Gerontol A Biol Sci Med Sci 61, 136-45
Zordan MA, Cisotto P, Benna C, Agostino A, Rizzo G, Piccin A, Pegoraro M, Sandrelli F, Perini G, Tognon G, De Caro R, Peron S, Kronniè TTe, Megighian A, Reggiani C, Zeviani M & Costa R (2006) Post-transcriptional silencing and functional characterization of the Drosophila melanogaster homolog of human Surf1. Genetics 172, 229-41
2005
Brand MD, Pakay JL, Ocloo A, Kokoszka J, Wallace DC, Brookes PS & Cornwall EJ (2005) The basal proton conductance of mitochondria depends on adenine nucleotide translocase content. Biochem J 392, 353-62
Greene JC, Whitworth AJ, Andrews LA, Parker TJ & Pallanck LJ (2005) Genetic and genomic studies of Drosophila parkin mutants implicate oxidative stress and innate immune responses in pathogenesis. Hum Mol Genet 14, 799-811
Jackson SM, Whitworth AJ, Greene JC, Libby RT, Baccam SL, Pallanck LJ & La Spada AR (2005) A SCA7 CAG/CTG repeat expansion is stable in Drosophila melanogaster despite modulation of genomic context and gene dosage. Gene 347, 35-41
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

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