title: Modeling mitochondrial encephalomyopathy in Drosophila.
abstract: Mitochondrial encephalomyopathies are disturbingly complex and devastating diseases, reflecting the underlying importance of the affected organelle. Therapeutic approaches for these diseases remain limited due to a poor understanding of disease pathogenesis resulting largely from a lack of tractable model systems in which to study these diseases. This is especially so for disease conditions resulting from mutations directly affecting the mitochondrial genome. Recent studies using Drosophila to develop genetic models with endogenous mitochondrial mutations suggest the fruit fly will contribute significantly to our understanding of mitochondrial disease pathogenesis and the development of novel therapeutic avenues.
authors.affiliation: Deparment of Pharmacology & Chemical Biology, University of Pittsburgh School of Medicine, Pittsburgh, PA 15261, USA. mjp44@pitt.edu
authors.forename: Michael J
authors.initials: MJ
authors.lastname: Palladino
mesh_terms: D000818:Animals; D004272:DNA, Mitochondrial / Q000235:genetics*; D004195:Disease Models, Animal; D004331:Drosophila melanogaster / Q000235:genetics* / Q000378:metabolism; D006801:Humans; D017237:Mitochondrial Encephalomyopathies / Q000235:genetics* / Q000378:metabolism / Q000503:physiopathology; D008957:Models, Genetic; D009154:Mutation / Q000235:genetics
chemical_list: D004272:DNA, Mitochondrial
references.citation: Exp Gerontol. 2010 Aug;45(7-8):489-502
references.pmid: 20123011
references.citation: Pediatr Neurol. 1995 Oct;13(3):242-6
references.pmid: 8554662
references.citation: J Child Neurol. 2001 Jul;16(7):533-5
references.pmid: 11453454
references.citation: Am J Med Genet A. 2007 Sep 1;143A(17):2046-51
references.pmid: 17663470
references.citation: PLoS One. 2010 Apr 07;5(4):e10054
references.pmid: 20383334
references.citation: Eur J Neurol. 2004 Mar;11(3):163-86
references.pmid: 15009163
references.citation: Cardiovasc Res. 2001 Jan;49(1):17-26
references.pmid: 11121792
references.citation: J Neurochem. 2008 Apr;105(1):165-76
references.pmid: 17999718
references.citation: Proc Natl Acad Sci U S A. 2006 Aug 1;103(31):11584-8
references.pmid: 16855048
references.citation: J Biol Chem. 2007 Apr 13;282(15):10853-64
references.pmid: 17261589
references.citation: Annu Rev Biochem. 1997;66:717-49
references.pmid: 9242922
references.citation: PLoS One. 2010 Jan 06;5(1):e8549
references.pmid: 20066047
references.citation: N Engl J Med. 1994 Mar 31;330(13):913-9
references.pmid: 8114864
references.citation: J Biol Chem. 2008 Apr 11;283(15):9749-58
references.pmid: 18252710
references.citation: J Biol Chem. 2010 Apr 9;285(15):11143-53
references.pmid: 20110367
references.citation: Am J Hum Genet. 1992 Apr;50(4):852-8
references.pmid: 1550128
references.citation: Brain Pathol. 2000 Jul;10(3):431-41
references.pmid: 10885662
references.citation: Biochim Biophys Acta. 2004 Dec 6;1659(2-3):190-6
references.pmid: 15576051
references.citation: Curr Opin Struct Biol. 2000 Dec;10(6):672-9
references.pmid: 11114504
references.citation: Biochim Biophys Acta. 1999 Feb 9;1410(2):99-102
references.pmid: 10084814
references.citation: Lancet. 2000 Jan 22;355(9200):299-304
references.pmid: 10675086
references.citation: J Neurosci. 2006 Jan 18;26(3):810-20
references.pmid: 16421301
references.citation: J Med Genet. 2009 Jan;46(1):64-7
references.pmid: 19124644
references.citation: Nature. 1999 Nov 18;402(6759):263-8
references.pmid: 10580496
references.citation: Biochim Biophys Acta. 2008 Jul-Aug;1777(7-8):592-8
references.pmid: 18485888
references.citation: Mitochondrion. 2003 Oct;3(2):83-96
references.pmid: 16120347
references.citation: Development. 2008 Aug;135(15):2669-79
references.pmid: 18599508
references.citation: Ann Clin Lab Sci. 2003 Fall;33(4):371-95
references.pmid: 14584751
references.citation: Am J Med Genet A. 2007 Apr 15;143A(8):808-16
references.pmid: 17352390
references.citation: Biochim Biophys Acta. 2006 Sep-Oct;1757(9-10):1190-8
references.pmid: 16806050
references.citation: Science. 2009 Oct 2;326(5949):144-7
references.pmid: 19797662
references.citation: Ann Neurol. 1993 Sep;34(3):410-2
references.pmid: 8395787
references.citation: Science. 1989 Apr 21;244(4902):346-9
references.pmid: 2711184
references.citation: Mol Cell Proteomics. 2008 May;7(5):995-1004
references.pmid: 18245802
references.citation: Lancet. 2000 Jan 29;355(9201):389-94
references.pmid: 10665569
references.citation: Cell. 1987 Oct 23;51(2):165-73
references.pmid: 3117373
references.citation: Nucleic Acids Res. 1990 Feb 11;18(3):561-7
references.pmid: 2308845
references.citation: Science. 2000 Aug 4;289(5480):782-5
references.pmid: 10926541
references.citation: N Engl J Med. 2003 Jun 26;348(26):2656-68
references.pmid: 12826641
references.citation: Nature. 1967 Jan 14;213(5072):137-9
references.pmid: 4291593
references.citation: Expert Opin Ther Targets. 2010 May;14(5):497-511
references.pmid: 20334487
references.citation: Methods Mol Biol. 2007;372:33-49
references.pmid: 18314716
references.citation: EMBO J. 2002 Feb 1;21(3):221-30
references.pmid: 11823415
references.citation: Annu Rev Pathol. 2010;5:297-348
references.pmid: 20078222
references.citation: J Physiol. 2004 Feb 15;555(Pt 1):1-13
references.pmid: 14660709
references.citation: Herz. 1994 Apr;19(2):105-18, 125
references.pmid: 8194831
references.citation: Biochim Biophys Acta. 1995 May 24;1271(1):221-7
references.pmid: 7599212
references.citation: Expert Opin Ther Targets. 2010 Apr;14(4):369-85
references.pmid: 20184395
references.citation: Science. 2008 Jul 25;321(5888):575-7
references.pmid: 18653897
references.citation: Neurology. 1997 Jul;49(1):270-3
references.pmid: 9222207
references.citation: Proc Natl Acad Sci U S A. 2010 Mar 16;107(11):5018-23
references.pmid: 20194754
references.citation: Am J Hum Genet. 1990 Mar;46(3):428-33
references.pmid: 2137962
references.citation: Proc Natl Acad Sci U S A. 2008 Dec 2;105(48):18735-9
references.pmid: 19020091
references.citation: J Bioenerg Biomembr. 1997 Apr;29(2):131-49
references.pmid: 9239539
references.citation: Dis Model Mech. 2009 Jul-Aug;2(7-8):336-40
references.pmid: 19553694
references.citation: EMBO J. 2008 Apr 9;27(7):1154-60
references.pmid: 18323778