The Alteration of Intrinsic Excitability and Synaptic Transmission in Lumbar Spinal Motor Neurons and Interneurons of Severe Spinal Muscular Atrophy Mice

Frontiers in Cellular Neuroscience
Jianli Sun, Melissa A Harrington

Abstract

Spinal muscular atrophy (SMA) is the leading genetic cause of death in infants. Studies with mouse models have demonstrated increased excitability and loss of afferent proprioceptive synapses on motor neurons (MNs). To further understand functional changes in the motor neural network occurring in SMA, we studied the intrinsic excitability and synaptic transmission of both MNs and interneurons (INs) from ventral horn in the lumbar spinal cord in the survival motor neuron (SMN)Δ7 mouse model. We found significant differences in the membrane properties of MNs in SMA mice compared to littermate controls, including hyperpolarized resting membrane potential, increased input resistance and decreased membrane capacitance. Action potential (AP) properties in MNs from SMA mice were also different from controls, including decreased rheobase current, increased amplitude and an increased afterdepolarization (ADP) potential. The relationship between AP firing frequency and injected current was reduced in MNs, as was the threshold current, while the percentage of MNs showing long-lasting potentiation (LLP) in the intrinsic excitability was higher in SMA mice. INs showed a high rate of spontaneous firing, and those from SMA mice fired at highe...Continue Reading

References

Aug 4, 1995·Biochemical and Biophysical Research Communications·M GennarelliB Dallapiccola
Apr 1, 1996·Neurobiology of Disease·T O Crawford, C A Pardo
May 26, 1999·Proceedings of the National Academy of Sciences of the United States of America·C L LorsonB Wirth
Jun 8, 1999·The Journal of Physiology·D FrickerR Miles
Apr 4, 2000·Physiological Reviews·J C ReklingJ L Feldman
Dec 9, 2000·Journal of Neurophysiology·P WhelanM J O'Donovan
Apr 3, 2001·Nature Reviews. Neuroscience·O Raineteau, M E Schwab
Aug 25, 2001·Annual Review of Neuroscience·A Reyes
Jul 2, 2002·Journal of Neurophysiology·David R Ireland, Wickliffe C Abraham
Oct 23, 2002·Proceedings of the National Academy of Sciences of the United States of America·Gael DaoudalDominique Debanne
Jan 28, 2003·Muscle & Nerve·Eric Beaumont, Phillip F Gardiner
Feb 19, 2003·Brain Research. Brain Research Reviews·Agnès BonnotMichael J O'Donovan
Dec 6, 2003·Learning & Memory·Gaël Daoudal, Dominique Debanne
May 1, 2004·Experimental Brain Research·Carl Y SaabStephen G Waxman
May 4, 2004·Experimental Cell Research·Amelie K GubitzGideon Dreyfuss
Feb 18, 2005·The Journal of Neuroscience : the Official Journal of the Society for Neuroscience·Jun XuJian Kang
Feb 25, 2005·The Journal of Neuroscience : the Official Journal of the Society for Neuroscience·David González-Forero, Francisco J Alvarez
Aug 27, 2005·The Journal of Physiology·Bruno CormeryPhillip Gardiner
Apr 8, 2006·Neuron·Hui-Juan HuRobert W Gereau
Jun 16, 2006·The Journal of Neuroscience : the Official Journal of the Society for Neuroscience·Guisheng ZhongRonald M Harris-Warrick
Mar 23, 2007·The Journal of Neuroscience : the Official Journal of the Society for Neuroscience·Miljen M JagodicSlobodan M Todorovic
May 22, 2007·Nature Reviews. Neuroscience·Bruce P Bean
Jul 10, 2007·Progress in Brain Research·Bryan C Hains, Stephen G Waxman
Mar 28, 2008·Neuron·Keiji IbataGina G Turrigiano
Apr 22, 2008·Nature Reviews. Neuroscience·Heinz Beck, Yoel Yaari
Jan 23, 2009·The Journal of Neuroscience : the Official Journal of the Society for Neuroscience·Lingling KongCharlotte J Sumner

❮ Previous
Next ❯

Citations


❮ Previous
Next ❯

Methods Mentioned

BETA
SMA

Software Mentioned

Clampfit
Excel
MiniAnalysis6

Related Concepts

Related Feeds

Cajal Bodies & Gems

Cajal bodies or coiled bodies are dense foci of coilin protein. Gemini of Cajal bodies, or gems, are microscopically similar to Cajal bodies. It is believed that Cajal bodies play important roles in RNA processing while gems assist the Cajal bodies. Find the latest research on Cajal bodies and gems here.