Neuromuscular transmission in a transgenic animal model of motor neuron disease

Yong I. Kim, Connie Joo, Charlie C. Cheng, Cristina E Davis, Thomas J. O'Shaughnessy

Research output: Chapter in Book/Report/Conference proceedingConference contribution

1 Citation (Scopus)

Abstract

The transgenic mice that express a human Cu,Zn superoxide dismutase (SOD) mutation are a plausible animal model of amyotrophic lateral sclerosis (ALS) in familial form. To explore the effects of the SOD mutation on motor nerve terminal (MNT) function, we studied neuromuscular transmission in mice with a mutated human SOD gene (G1H and G1L) as well as mice with the wild-type human SOD gene (N29) and normal untreated (wild-type, wt) animals for use as controls. In both G1H and G1L animals, the quantal content (m) of the neurally-evoked end-plate potentials (EPPs) was significantly increased from the values found in the corresponding control mice. The frequency and amplitude of the spontaneously occurring miniature end-plate potentials (MEPPs) were not significantly different, however. This finding is interpreted as indicating that calcium entry into the MNT, presumably mediated by the voltage-dependent calcium channels, is significantly increased by the mutation. This is consistent with the hypothesis that the Ca 2+-mediated neurotoxicity causes degeneration of motor neurons in this disorder.

Original languageEnglish (US)
Title of host publicationAnnual International Conference of the IEEE Engineering in Medicine and Biology - Proceedings
PublisherIEEE
Pages1773-1774
Number of pages2
Volume4
StatePublished - 1996
Externally publishedYes
EventProceedings of the 1996 18th Annual International Conference of the IEEE Engineering in Medicine and Biology Society. Part 4 (of 5) - Amsterdam, Neth
Duration: Oct 31 1996Nov 3 1996

Other

OtherProceedings of the 1996 18th Annual International Conference of the IEEE Engineering in Medicine and Biology Society. Part 4 (of 5)
CityAmsterdam, Neth
Period10/31/9611/3/96

Fingerprint

Neurons
Superoxide Dismutase
Animals
Genes
Calcium
Calcium Channels
Electric potential

ASJC Scopus subject areas

  • Bioengineering

Cite this

Kim, Y. I., Joo, C., Cheng, C. C., Davis, C. E., & O'Shaughnessy, T. J. (1996). Neuromuscular transmission in a transgenic animal model of motor neuron disease. In Annual International Conference of the IEEE Engineering in Medicine and Biology - Proceedings (Vol. 4, pp. 1773-1774). IEEE.

Neuromuscular transmission in a transgenic animal model of motor neuron disease. / Kim, Yong I.; Joo, Connie; Cheng, Charlie C.; Davis, Cristina E; O'Shaughnessy, Thomas J.

Annual International Conference of the IEEE Engineering in Medicine and Biology - Proceedings. Vol. 4 IEEE, 1996. p. 1773-1774.

Research output: Chapter in Book/Report/Conference proceedingConference contribution

Kim, YI, Joo, C, Cheng, CC, Davis, CE & O'Shaughnessy, TJ 1996, Neuromuscular transmission in a transgenic animal model of motor neuron disease. in Annual International Conference of the IEEE Engineering in Medicine and Biology - Proceedings. vol. 4, IEEE, pp. 1773-1774, Proceedings of the 1996 18th Annual International Conference of the IEEE Engineering in Medicine and Biology Society. Part 4 (of 5), Amsterdam, Neth, 10/31/96.
Kim YI, Joo C, Cheng CC, Davis CE, O'Shaughnessy TJ. Neuromuscular transmission in a transgenic animal model of motor neuron disease. In Annual International Conference of the IEEE Engineering in Medicine and Biology - Proceedings. Vol. 4. IEEE. 1996. p. 1773-1774
Kim, Yong I. ; Joo, Connie ; Cheng, Charlie C. ; Davis, Cristina E ; O'Shaughnessy, Thomas J. / Neuromuscular transmission in a transgenic animal model of motor neuron disease. Annual International Conference of the IEEE Engineering in Medicine and Biology - Proceedings. Vol. 4 IEEE, 1996. pp. 1773-1774
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