Seizures, enhanced excitation, and increased vesicle number in Lis1 mutant mice.

Annals of Neurology
Joel S F GreenwoodScott C Baraban

Abstract

In humans, abnormal neuronal migration and severe neuronal disorganization resulting from Lis1 (lissencephaly) haploinsufficiency contributes to cognitive impairment and seizures early in life. In Lis1 heterozygotic mice, severe hippocampal disorganization and cognitive impairment have also been reported. Using this mouse model, we examined the functional impact of LIS1 deficiency with particular focus on excitatory glutamate-mediated synaptic transmission. We used visualized patch-clamp recordings in acute hippocampal slices. We recorded spontaneous, miniature and stimulation-evoked excitatory postsynaptic current (EPSC). Additional mice were processed for immunohistochemistry, electron microscopy (EM), or video-electroencephalographic (EEG) monitoring. Video-EEG confirmed the presence of spontaneous electrographic seizures in Lis1 mutant mice. In disorganized hippocampal slices from Lis1(+/-) mice, we noted a nearly two-fold significant increase in the frequency of spontaneous and miniature EPSC; no significant change in amplitude or decay was noted. Synaptic function assessed using brief repetitive or paired-pulse stimulation protocols, also revealed significant enhancement of glutamate-mediated excitation. Low concentration...Continue Reading

References

Jun 1, 1995·Neuropediatrics·W B Dobyns, C L Truwit
Sep 1, 1996·Epilepsia·R D TraubJ G Jefferys
Apr 1, 1996·The European Journal of Neuroscience·Y PerezJ C Lacaille
Dec 14, 1999·Learning & Memory·R PaylorA Wynshaw-Boris
Jan 15, 2000·Journal of Neurophysiology·R A DeFazio, J J Hablitz
Jul 6, 2000·Journal of Neuroscience Research·J Y LiA Dahlström
Feb 5, 2002·Annual Review of Physiology·Robert S Zucker, Wade G Regehr
Aug 30, 2002·Epilepsy Research·Matthew D SmythScott C Baraban
Feb 16, 1965·Proceedings of the Royal Society of London. Series B, Containing Papers of a Biological Character·B KATZ, R MILEDI
Apr 1, 2004·Cerebral Cortex·Véronique M AndréGary W Mathern
Sep 24, 2004·Journal of Neurophysiology·K M Jacobs, D A Prince
Jan 28, 2005·The Journal of Neuroscience : the Official Journal of the Society for Neuroscience·Veronica A Ledoux, Catherine S Woolley
Sep 8, 2005·The Journal of Cell Biology·Jin-Wu TsaiRichard B Vallee
Oct 21, 2005·The Journal of Neuroscience : the Official Journal of the Society for Neuroscience·Steven A KushnerAlcino J Silva
Jul 13, 2006·Epilepsy Research·Wolfgang Löscher, Dieter Schmidt
Sep 8, 2006·Journal of Neuropathology and Experimental Neurology·Adele FinardiGiorgio Battaglia
Nov 18, 2006·Proceedings of the National Academy of Sciences of the United States of America·Ruud F G ToonenMatthijs Verhage
May 4, 2007·Journal of Neurophysiology·A Zsombok, K M Jacobs
Sep 21, 2007·Journal of Neurophysiology·Daniel L Jones, Scott C Baraban
Oct 4, 2007·Epilepsia·Lynda El-HassarChristophe Bernard
Nov 6, 2007·Nature Neuroscience·Edward GlasscockJeffrey L Noebels

❮ Previous
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Citations

Oct 27, 2012·Journal of Neurophysiology·Joy Y SebeScott C Baraban
Aug 17, 2012·The Journal of Neuroscience : the Official Journal of the Society for Neuroscience·Matthew C WestonJohn W Swann
Sep 14, 2012·The Journal of Neuroscience : the Official Journal of the Society for Neuroscience·Robert F HuntScott C Baraban
Nov 25, 2011·The Journal of Neuroscience : the Official Journal of the Society for Neuroscience·Jai P Pandey, Deanna S Smith
Jun 17, 2011·The Journal of Neuroscience : the Official Journal of the Society for Neuroscience·Nicholas J BradshawJ Kirsty Millar
Mar 14, 2013·EMBO Molecular Medicine·Anamaria SudarovMargaret Elizabeth Ross
Apr 26, 2015·Journal of Neuroscience Methods·Michael Wong, Steven N Roper
Oct 31, 2015·Current Opinion in Neurobiology·Meagan S Siehr, Jeffrey L Noebels
Nov 26, 2011·Pediatric Neurology·Alex R PaciorkowskiWilliam B Dobyns
Mar 13, 2016·Cellular and Molecular Life Sciences : CMLS·Dieudonnée van de WilligeAnna Akhmanova
Aug 25, 2015·Neurobiology of Disease·Melissa A StoufferFiona Francis
Mar 14, 2014·Frontiers in Cellular Neuroscience·Richard BelvindrahFiona Francis
Oct 12, 2016·Nihon yakurigaku zasshi. Folia pharmacologica Japonica·Yosuke Kitahara, Akinori Nishi
May 23, 2020·Cells·Amy J Tibbo, George S Baillie
Aug 31, 2020·Cerebral Cortex Communications·Cezar GavriloviciMinh Dang Nguyen
May 23, 2012·Chemical Reviews·Ortrud K Steinlein
Feb 2, 2022·Brain Structure & Function·E Domínguez-SalaEmilio Geijo-Barrientos

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