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 language | English (US) |
|---|---|
| Title of host publication | Annual International Conference of the IEEE Engineering in Medicine and Biology - Proceedings |
| Publisher | IEEE |
| Pages | 1773-1774 |
| Number of pages | 2 |
| Volume | 4 |
| State | Published - 1996 |
| Externally published | Yes |
| Event | Proceedings 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 1996 → Nov 3 1996 |
Other
| Other | Proceedings of the 1996 18th Annual International Conference of the IEEE Engineering in Medicine and Biology Society. Part 4 (of 5) |
|---|---|
| City | Amsterdam, Neth |
| Period | 10/31/96 → 11/3/96 |
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ASJC Scopus subject areas
- Bioengineering
Cite this
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 proceeding › Conference contribution
}
TY - GEN
T1 - Neuromuscular transmission in a transgenic animal model of motor neuron disease
AU - Kim, Yong I.
AU - Joo, Connie
AU - Cheng, Charlie C.
AU - Davis, Cristina E
AU - O'Shaughnessy, Thomas J.
PY - 1996
Y1 - 1996
N2 - 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.
AB - 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.
UR - http://www.scopus.com/inward/record.url?scp=0030314515&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=0030314515&partnerID=8YFLogxK
M3 - Conference contribution
AN - SCOPUS:0030314515
VL - 4
SP - 1773
EP - 1774
BT - Annual International Conference of the IEEE Engineering in Medicine and Biology - Proceedings
PB - IEEE
ER -