GABAergic miniature spontaneous activity is increased in the CA1 hippocampal region of dystrophic mdx mice

Neuromuscular Disorders : NMD
Laura GraciottiGianluca Fulgenzi

Abstract

Duchenne muscular dystrophy (DMD), a genetic disease due to dystrophin gene mutation and characterised by skeletal muscle failure, is associated with non-progressive cognitive deficits. In human and mouse brain, full-length dystrophin is localised postsynaptically in neocortical, hippocampal and cerebellar neurons. Evidence obtained in the CNS of dystrophic mice (mdx) suggested alterations of the GABAergic system. However, a direct functional evaluation of GABAergic synaptic transmission in mdx mice has not been conducted in the hippocampus, which is involved in cognitive processes and is rich in full-length dystrophin. Here, we investigated evoked and miniature inhibitory postsynaptic currents (IPSCs) in CA1 pyramidal neurons of mdx mice with patch clamp recording techniques. Results showed an increased frequency of miniature spontaneous IPSCs in mdx mice compared with controls, whereas evoked IPSCs did not show significant variations. Paired-pulse facilitation (PPF) analysis showed lack of facilitation at short intervals in mdx mice compared with that in wild-type mice. Analysis of density of synapses that innervate CA1 pyramidal cell bodies did not indicate significant differences between mdx mice and controls. Therefore, we...Continue Reading

References

Dec 1, 1992·Proceedings of the National Academy of Sciences of the United States of America·T W KimI B Black
Jan 1, 1991·Neuromuscular Disorders : NMD·F MuntoniG Serra
Sep 20, 1991·Cell·J M Ervasti, K P Campbell
Dec 1, 1989·Journal of Neuroscience Methods·M G BlantonA R Kriegstein
Jun 1, 1986·Journal of Microscopy·A J BaddeleyL M Cruz-Orive
Jan 22, 1994·The Journal of Comparative Neurology·C BeaulieuC Crevier
Nov 10, 1995·Neuroscience Letters·A SbriccoliD Minciacchi
Apr 10, 1999·Neuron·S SugitaT C Südhof
Jul 27, 2001·The Journal of Cell Biology·S SugitaT C Südhof
Dec 12, 2001·Neuromuscular Disorders : NMD·Caroline RaeStewart Head
Feb 9, 2002·Brain : a Journal of Neurology·J L AndersonJ W Morley
Aug 14, 2003·Brain Research·Jennifer L AndersonJohn W Morley
Aug 13, 2004·Brain Research·Jennifer L AndersonJohn W Morley
Jun 1, 1965·Archives of Disease in Childhood·V Dubowitz

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Citations

Nov 10, 2009·Molecular Neurobiology·Gonneke S K PilgramJasprina N Noordermeer
Feb 22, 2012·Human Molecular Genetics·Caroline PerronnetCyrille Vaillend
Jul 14, 2010·Journal of Biomedicine & Biotechnology·Caroline Perronnet, Cyrille Vaillend
Jan 28, 2009·Molecular and Cellular Neurosciences·Glen B BanksStanley C Froehner
Sep 22, 2012·Brain Research·Paulo César GhediniCaden Souccar
Feb 14, 2015·Neuroscience and Biobehavioral Reviews·Ruben G F HendriksenMarlien W Aalbers
Dec 3, 2014·Brain Research Bulletin·Erez James CohenDiego Minciacchi
Jul 28, 2016·Frontiers in Cellular Neuroscience·Ruben G F HendriksenJohan S H Vles
Mar 6, 2015·Disease Models & Mechanisms·Joe W McGreevyDongsheng Duan

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