title: Drosophila modeling of heritable neurodevelopmental disorders.
abstract: Heritable neurodevelopmental disorders are multifaceted disease conditions encompassing a wide range of symptoms including intellectual disability, cognitive dysfunction, autism and myriad other behavioral impairments. In cases where single, causative genetic defects have been identified, such as Angelman syndrome, Rett syndrome, Neurofibromatosis Type 1 and Fragile X syndrome, the classical Drosophila genetic system has provided fruitful disease models. Recent Drosophila studies have advanced our understanding of UBE3A, MECP2, NF1 and FMR1 function, respectively, in genetic, biochemical, anatomical, physiological and behavioral contexts. Investigations in Drosophila continue to provide the essential mechanistic understanding required to facilitate the conception of rational therapeutic treatments.
authors: Departments of Biological Sciences and Cell and Developmental Biology, Kennedy Center for Research on Human Development, Vanderbilt University, Nashville, TN 37232, USA.
authors: Cheryl L
authors: CL
authors: Gatto
authors: Kendal
authors: K
authors: Broadie
references: Annu Rev Neurosci. 2010;33:221-43
references: 20345245
references: Dis Model Mech. 2010 Nov-Dec;3(11-12):693-700
references: 20682752
references: Am J Med Genet A. 2009 Feb 15;149A(4):722-5
references: 19253388
references: Hum Mol Genet. 2005 Jul 15;14(14):1935-46
references: 15917271
references: Neurobiol Dis. 2011 Mar;41(3):669-77
references: 21147225
references: Hum Mol Genet. 2003 Apr 15;12(8):837-47
references: 12668607
references: Nat Neurosci. 2008 Oct;11(10):1143-5
references: 18776892
references: J Neurosci. 2008 Mar 19;28(12):3221-6
references: 18354025
references: J Neurosci. 2010 Jul 28;30(30):10135-43
references: 20668197
references: Science. 1997 May 2;276(5313):791-4
references: 9115203
references: Horm Behav. 2011 Mar;59(3):364-8
references: 20515694
references: Genet Med. 2010 Jul;12(7):385-95
references: 20445456
references: Front Synaptic Neurosci. 2010 Jun 07;2:4
references: 21423490
references: Mol Neurobiol. 2009 Apr;39(2):107-29
references: 19214804
references: Dev Biol. 2010 Apr 15;340(2):408-18
references: 20122915
references: Neurobiol Dis. 2010 Jul;39(1):13-20
references: 20304067
references: PLoS Genet. 2009 Jun;5(6):e1000536
references: 19557195
references: Curr Biol. 2010 Aug 24;20(16):1487-92
references: 20691595
references: Nature. 2009 May 28;459(7246):569-73
references: 19404257
references: Proc Natl Acad Sci U S A. 2005 Jun 14;102(24):8573-8
references: 15937108
references: Neurobiol Dis. 2010 Dec;40(3):586-92
references: 20696245
references: Am J Med Genet A. 2010 May;152A(5):1079-88
references: 20425814
references: J Med Genet. 2008 Aug;45(8):498-9
references: 18413371
references: Curr Biol. 2005 Jun 21;15(12):1156-63
references: 15964283
references: Hum Mol Genet. 2006 Sep 15;15(18):2825-35
references: 16905559
references: Genet Med. 2006 Dec;8(12):784-92
references: 17172942
references: PLoS Genet. 2009 Apr;5(4):e1000444
references: 19343200
references: Cell. 2001 Nov 30;107(5):591-603
references: 11733059
references: PLoS One. 2009 Oct 27;4(10):e7618
references: 19888420
references: J Neurosci. 2007 Jun 20;27(25):6852-7
references: 17581973
references: J Neurosci. 2009 Feb 18;29(7):1948-61
references: 19228950
references: Am J Hum Genet. 2008 Jul;83(1):89-93
references: 18571142
references: Science. 1997 May 2;276(5313):795-8
references: 9115204
references: Neuron. 2002 Jun 13;34(6):973-84
references: 12086644
references: J Comp Physiol A. 1985 Sep;157(2):263-77
references: 3939242
references: Front Neural Circuits. 2010 Jan 08;3:22
references: 20190856
references: Dev Biol. 2009 Jun 1;330(1):83-92
references: 19306863
references: J Neurosci. 2010 May 12;30(19):6782-92
references: 20463240
references: Curr Psychiatry Rep. 2010 Apr;12(2):127-34
references: 20425298
references: Brain Dev. 2011 Sep;33(8):627-31
references: 21232889
references: Cell. 2008 Oct 31;135(3):549-60
references: 18984165
references: Hum Mol Genet. 2010 Aug 1;19(15):3068-79
references: 20504994
references: Proc Natl Acad Sci U S A. 2008 Aug 26;105(34):12399-404
references: 18701717
references: Genetics. 2009 Aug;182(4):1051-60
references: 19487564
references: Eur J Hum Genet. 2009 Dec;17(12):1577-81
references: 19623215
references: Am J Ment Retard. 2008 Nov;113(6):427-38
references: 19127654
references: Trends Genet. 2003 May;19(5):269-77
references: 12711219
references: Curr Biol. 2004 Jul 13;14(13):1135-47
references: 15242610
references: Science. 2001 Sep 21;293(5538):2251-6
references: 11567138
references: Epilepsia. 2009 Nov;50(11):2331-9
references: 19874386
references: Genome Res. 2001 Jun;11(6):1114-25
references: 11381037
references: Dis Model Mech. 2010 Jul-Aug;3(7-8):471-85
references: 20442204
references: Biogerontology. 2010 Jun;11(3):347-62
references: 20039205
references: Nature. 2000 Feb 24;403(6772):895-8
references: 10706287
references: PLoS Genet. 2008 Sep 05;4(9):e1000179
references: 18773074
references: Neurosci Biobehav Rev. 2008 Oct;32(8):1533-43
references: 18601949
references: Fly (Austin). 2010 Jul-Sep;4(3):216-25
references: 20519966
references: J Med Genet. 2010 Jan;47(1):49-53
references: 19578037
references: Front Neural Circuits. 2009 Aug 20;3:8
references: 19738924
references: Cell. 1994 Jul 15;78(1):23-33
references: 8033209
references: J Clin Neurosci. 2001 Sep;8(5):421-2
references: 11535008
references: Front Mol Neurosci. 2009 Jul 30;2:8
references: 19668708
references: Pract Neurol. 2010 Apr;10(2):82-93
references: 20308235
references: Hum Mol Genet. 2011 Jan 1;20(1):51-63
references: 20935173
references: Curr Biol. 2005 Sep 6;15(17):R700-13
references: 16139203
references: Development. 2010 Dec;137(24):4201-9
references: 21068064
references: Ann Neurol. 2009 Dec;66(6):771-82
references: 20035514
references: Hum Mol Genet. 2009 Feb 1;18(3):454-62
references: 18996915
references: Am J Hum Genet. 2005 Sep;77(3):442-53
references: 16080119
references: Mol Cell Proteomics. 2005 Mar;4(3):278-90
references: 15634690
references: J Neurol Neurosurg Psychiatry. 2010 Mar;81(3):304-9
references: 20185469
references: Neurobiol Dis. 2011 Jan;41(1):147-59
references: 20843478
references: Neuron. 2005 Mar 3;45(5):753-64
references: 15748850
references: J Med Genet. 2005 Feb;42(2):e12
references: 15689435