title: Origins of epilepsy in fragile X syndrome.
abstract: Fragile X syndrome is the leading heritable form of cognitive impairment and the leading known monogenic disorder associated with autism. Roughly one-quarter of children with this disorder have seizures, most of which are relatively benign and are resolved beyond childhood. Because of the prevalence of fragile X syndrome, numerous animal models have been developed and electrophysiological studies have taken place to investigate its pathogenesis. The investigations have yielded a wealth of information regarding the synaptic dysfunction that underlies the hyperexcitability and epileptiform features associated with this disorder.
authors.affiliation: Department of Biochemistry and Molecular Medicine, University of California, School of Medicine, Davis, California, USA. pjhagerman@ucdavis.edu
authors.forename: Paul J
authors.initials: PJ
authors.lastname: Hagerman
authors.forename: Carl E
authors.initials: CE
authors.lastname: Stafstrom
references.citation: Proc Natl Acad Sci U S A. 1997 May 13;94(10):5395-400
references.pmid: 9144248
references.citation: Nat Chem Biol. 2008 Apr;4(4):256-63
references.pmid: 18327252
references.citation: Am J Med Genet B Neuropsychiatr Genet. 2009 Sep 5;150B(6):782-98
references.pmid: 19105204
references.citation: Genes Brain Behav. 2004 Dec;3(6):337-59
references.pmid: 15544577
references.citation: Cell. 2001 Nov 30;107(5):591-603
references.pmid: 11733059
references.citation: J Neurosci. 2005 Aug 31;25(35):8048-55
references.pmid: 16135762
references.citation: J Dev Behav Pediatr. 2008 Aug;29(4):293-302
references.pmid: 18698192
references.citation: Trends Neurosci. 2004 Jul;27(7):370-7
references.pmid: 15219735
references.citation: Am J Med Genet A. 2004 Sep 1;129A(3):225-34
references.pmid: 15326621
references.citation: J Neurophysiol. 2008 Nov;100(5):2615-26
references.pmid: 18784272
references.citation: Science. 2000 May 19;288(5469):1254-7
references.pmid: 10818003
references.citation: J Med Genet. 2009 Apr;46(4):266-71
references.pmid: 19126569
references.citation: Trends Neurosci. 2007 Aug;30(8):425-31
references.pmid: 17590448
references.citation: Dev Med Child Neurol. 2002 Nov;44(11):724-8
references.pmid: 12418611
references.citation: Proc Natl Acad Sci U S A. 2008 Mar 18;105(11):4429-34
references.pmid: 18332424
references.citation: Neurosci Lett. 2007 Feb 2;412(3):227-32
references.pmid: 17197085
references.citation: Proc Natl Acad Sci U S A. 2002 May 28;99(11):7746-50
references.pmid: 12032354
references.citation: Am J Med Genet A. 2008 Aug 1;146A(15):1911-6
references.pmid: 18627038
references.citation: Neurobiol Dis. 2006 Feb;21(2):346-57
references.pmid: 16199166
references.citation: Cereb Cortex. 2009 Jul;19(7):1504-14
references.pmid: 18832330
references.citation: J Neurosci. 2009 Mar 18;29(11):3497-507
references.pmid: 19295155
references.citation: J Med Genet. 2008 Aug;45(8):498-9
references.pmid: 18413371
references.citation: Neuron. 2007 Dec 20;56(6):955-62
references.pmid: 18093519
references.citation: Neuroscientist. 2009 Oct;15(5):549-67
references.pmid: 19325170
references.citation: Pediatrics. 2009 Jan;123(1):378-90
references.pmid: 19117905
references.citation: J Neurophysiol. 2000 May;83(5):2844-53
references.pmid: 10805682
references.citation: J Neurochem. 2007 Apr;101(2):411-21
references.pmid: 17402970
references.citation: J Neurosci. 2004 Jan 7;24(1):76-84
references.pmid: 14715940
references.citation: Proc Natl Acad Sci U S A. 2007 Sep 25;104(39):15537-42
references.pmid: 17881561
references.citation: Health Technol Assess. 2003;7(16):1-106
references.pmid: 12969542
references.citation: Neuron. 2008 Oct 23;60(2):201-14
references.pmid: 18957214
references.citation: Lancet Neurol. 2007 Jan;6(1):45-55
references.pmid: 17166801
references.citation: Brain Res. 2009 Feb 9;1253:176-83
references.pmid: 19070606
references.citation: Ment Retard Dev Disabil Res Rev. 2004;10(1):31-41
references.pmid: 14994286
references.citation: Neuropharmacology. 1999 Oct;38(10):1493-503
references.pmid: 10530811
references.citation: J Dev Behav Pediatr. 2006 Feb;27(1):63-74
references.pmid: 16511373
references.citation: J Neurophysiol. 2006 May;95(5):3291-5
references.pmid: 16452252
references.citation: Neurotherapeutics. 2009 Apr;6(2):392-401
references.pmid: 19332335
references.citation: Neurosci Lett. 2005 Apr 4;377(3):141-6
references.pmid: 15755515
references.citation: Dev Cell. 2008 Jun;14(6):926-39
references.pmid: 18539120
references.citation: J Neurosci. 2002 May 1;22(9):3795-805
references.pmid: 11978855
references.citation: Hum Mol Genet. 2006 Dec 1;15(23):3446-58
references.pmid: 17065172
references.citation: J Neurosci. 2001 Aug 15;21(16):6387-94
references.pmid: 11487662
references.citation: Neuropharmacology. 2005 Dec;49(7):1053-66
references.pmid: 16054174
references.citation: Brain Dev. 2002 Dec;24(8):766-9
references.pmid: 12453600
references.citation: Brain Res. 2006 Nov 22;1121(1):238-45
references.pmid: 17046729
references.citation: Neuron. 2005 Mar 3;45(5):753-64
references.pmid: 15748850
references.citation: Cereb Cortex. 2009 Jul;19(7):1515-20
references.pmid: 18787232
references.citation: Nat Rev Neurosci. 2005 May;6(5):376-87
references.pmid: 15861180