Altered excitability of motor neurons in a transgenic mouse model of familial amyotrophic lateral sclerosis

Neuroscience Letters

Volumn 351, Issue 3, 2003, Pages 153-156

  Pieri, Massimo ^a   Albo, Federica ^b   Gaetti, Chiara ^c   Spalloni, Alida ^b   Bengtson, C Peter ^b   Longone, Patrizia ^b   Cavalcanti, Silvio ^c   Zona, Cristina ^a,b  

^a UNIVERSITY OF ROME TOR VERGATA   (Italy)

^b IRCCS FONDAZIONE SANTA LUCIA   (Italy)

^c UNIVERSITY OF BOLOGNA   (Italy)

Author keywords

Action potentials; Cellular culture; Excitability; G93A mice; Motor neurons; Patch clamp; Superoxide dismutase

Indexed keywords

ALANINE; COPPER ZINC SUPEROXIDE DISMUTASE; GLYCINE;

ACTION POTENTIAL; AMINO ACID SUBSTITUTION; AMYOTROPHIC LATERAL SCLEROSIS; ANIMAL CELL; ARTICLE; CONTROLLED STUDY; ELECTROPHYSIOLOGY; EMBRYO; FAMILIAL DISEASE; GENE MUTATION; IMMUNOHISTOCHEMISTRY; MEMBRANE STEADY POTENTIAL; MOUSE; NERVE CELL EXCITABILITY; NONHUMAN; PATCH CLAMP; PRIORITY JOURNAL; SPINAL CORD MOTONEURON; TRANSGENIC MOUSE;

EID: 0242416191     PISSN: 03043940     EISSN: None     Source Type: Journal    
DOI: 10.1016/j.neulet.2003.07.010     Document Type: Article

References (20)

1. Caramia M.D., Palmieri M.G., Desiato M.T., Iani C., Scalise A., Telera S., Bernardi G. Pharmacologic reversal of cortical hyperexcitability in patients with ALS. Neurology. 54:(1):2000;58-64.
2. Carriedo S.G., Yin H.Z., Weiss J.H.J. Motor neurons are selectively vulnerable to AMPA/kainate receptor-mediated injury in vitro. J. Neurosci. 16:1996;4069-4079.
3. Carriedo S.G., Yin H.Z., Sensi S.L., Weiss J.H. Rapid Ca2+ entry through Ca2+-permeable AMPA/kainate channels triggers marked intracellular Ca2+ rises and consequent oxygen radical production. J. Neurosci. 18:1998;7727-7738.
4. Estevez A.G., Crow J.P., Sampson J.B., Reiter C., Zhuang Y., Richardson G.J., Tarpey M.M., Barbeito L., Beckman J.S. Induction of nitric oxide-dependent apoptosis in motor neurons by zinc-deficient superoxide dismutase. Science. 286:(5449):1999;2498-2500.
5. Gao B.X., Ziskind-Conhaim L. Development of ionic currents underlying changes in action potential waveforms in rat spinal motoneurons. J. Neurophysiol. 80:1998;3047-3061.
6. Geracitano R., Paolucci E., Prisco S., Guatteo E., Zona C., Longone P., Ammassari-Teule M., Bernardi G., Berretta N., Mercuri N.B. Altered long-term corticostriatal synaptic plasticity in transgenic mice overexpressing human CU/ZN superoxide dismutase (GLY(93)→ALA) mutation. Neuroscience. 118:(2):2003;399-408.
7. Hess D., El Manira A. Characterization of a high-voltage-activated IA current with a role in spike timing and locomotor pattern generation. Proc. Natl. Acad. Sci. USA. 98:2001;5276-5281.
8. Higgins C.M., Jung C., Ding H., Xu Z. Mutant Cu, Zn superoxide dismutase that causes motoneuron degeneration is present in mitochondria in the CNS. J. Neurosci. 22:2002;RC215.
9. Johnston J.A., Dalton M.J., Gurney M.E., Kopito R.R. Formation of high molecular weight complexes of mutant Cu, Zn-superoxide dismutase in a mouse model for familial amyotrophic lateral sclerosis. Proc. Natl. Acad. Sci. USA. 97:2000;12571-12576.
10. Mills K.R., Nithi K.A. Corticomotor threshold is reduced in early sporadic amyotrophic lateral sclerosis. Muscle Nerve. 20:1997;1137-1141.
11. Naka D., Mills K.R. Further evidence for corticomotor hyperexcitability in amyotrophic lateral sclerosis. Muscle Nerve. 23:2000;1044-1050.
12. Pramatarova A., Figlewicz D.A., Krizus A., Ceballos Picot I., Nicole A., Dib M., Meininger V., Brown R.H., Rouleau G.A. Identification of new mutations in the Cu/Zn superoxide dismutase gene of patients with familial amyotrophic lateral sclerosis. Am. J. Hum. Genet. 56:1995;592-596.
13. Rigo A., Viglino P., Rotilio G. Polarographic determination of superoxide dismutase. Anal. Biochem. 68:1975;1-8.
14. Rosen D.R., Siddique T., Patterson D., Figlewicz D.A., Sapp P., Hentati A., Donaldson D., Goto J., Regan J.P., Deng H.X. Mutations in Cu/Zn superoxide dismutase gene are associated with familial amyotrophic lateral sclerosis. Nature. 362:1993;59-62.
15. Vandenberge W., Robberecht W., Brorson J.R. AMPA receptor calcium permeability, GluR2 expression, and selective motoneuron vulnerability. J. Neurosci. 20:2000;123-132.
16. Van Den Bosch L., Vandenberghe W., Klaassen H., Van Houtte E., Robberecht W. Ca(2+)-permeable AMPA receptors and selective vulnerability of motor neurons. J. Neurol. Sci. 180:2000;29-34.
17. Weiss J.H., Sensi S.L. Ca2+-Zn2+ permeable AMPA or kainate receptors: possible key factors in selective neurodegeneration. Trends Neurosci. 23:2000;365-371.
18. Zanette G., Tamburin S., Manganotti P., Refatti N., Forgione A., Rizzuto N. Different mechanisms contribute to motor cortex hyperexcitability in amyotrophic lateral sclerosis. Clin. Neurophysiol. 113:(11):2002;1688-1697.
19. Zona C., Ferri A., Gabbianelli R., Mercuri N.B., Bernardi G., Rotilio G., Carrì M.T. Voltage-activated sodium currents in a cell line expressing a Cu, Zn superoxide dismutase typical of familial ALS. NeuroReport. 9:1998;3515-3518.
20. Zona C., Siniscalchi A., Mercuri N.B., Bernardi G. Riluzole interacts with voltage-activated sodium and potassium currents in cultured rat cortical neurons. Neuroscience. 85:1998;931-938.