Hyperexcitability of Cultured Spinal Motoneurons from Presymptomatic ALS Mice

Journal of Neurophysiology

Volumn 91, Issue 1, 2004, Pages 571-575

  Kuo, Jason J ^a   Schonewille, Martijn ^b   Siddique, Teepu ^a   Schults, Annet N A ^b   Fu, Ronggen ^a   Bär, Peter R ^b   Anelli, Roberta ^a   Heckman, C J ^a   Kroese, Alfons B A ^b  

^a NORTHWESTERN UNIVERSITY   (United States)

^b UNIVERSITY MEDICAL CENTER UTRECHT   (Netherlands)

Author keywords

[No Author keywords available]

Indexed keywords

COPPER ZINC SUPEROXIDE DISMUTASE;

ACTION POTENTIAL; AMYOTROPHIC LATERAL SCLEROSIS; ANIMAL EXPERIMENT; ANIMAL MODEL; ANIMAL TISSUE; ARTICLE; CELL CULTURE; CONTROLLED STUDY; DISEASE MODEL; ELECTROPHYSIOLOGY; EMBRYO; GENE OVEREXPRESSION; MEMBRANE STEADY POTENTIAL; MOTOR NERVE CONDUCTION; MOUSE; NERVE EXCITABILITY; NEWBORN; NONHUMAN; PATHOGENESIS; PRIORITY JOURNAL; SPINAL CORD MOTONEURON; SYNAPTIC TRANSMISSION; TRANSGENIC MOUSE;

ACTION POTENTIALS; ALANINE; AMYOTROPHIC LATERAL SCLEROSIS; ANIMALS; ANIMALS, NEWBORN; DISEASE MODELS, ANIMAL; ELECTRIC IMPEDANCE; ELECTRIC STIMULATION; EMBRYO; GLYCINE; LINEAR MODELS; MICE; MICE, TRANSGENIC; MOTOR NEURONS; SPINAL CORD; SUPEROXIDE DISMUTASE;

EID: 0345827415     PISSN: 00223077     EISSN: None     Source Type: Journal    
DOI: 10.1152/jn.00665.2003     Document Type: Article

References (30)

1. Anelli R, Dunn ME, and Mugnaini E. Unipolar brush cells develop a set of characteristic features in primary cerebellar cultures. J Neurocytol 29: 129-144, 2000.
2. Bär PR. Motor neuron disease in vitro: the use of cultured motor neurons to study amyotrophic lateral sclerosis. Eur J Pharmacol 405: 285-295, 2000.
3. Bruijn LI, Houseweart MK, Kato S, Anderson KL, Anderson SD, Ohama E, Reaume AG, Scott RW, and Cleveland DW. Aggregation and motor neuron toxicity of an ALS-linked SOD1 mutant independent from wild-type SOD1. Science 281: 1851-1854, 1998.
4. 2+ dependent. Neuroreport 6: 945-948, 1995.
5. Cleveland DW and Rothstein JD. From Charcot to Lou Gehrig: deciphering selective motor neuron death in ALS. Nat Rev Neurosci 2: 806-819, 2001.
6. Eisen A and Weber M. The motor cortex and amyotrophic lateral sclerosis. Muscle Nerve 24: 564-573, 2001.
7. Ellis DZ, Rabe J, and Sweadner KJ. Global loss of Na, K-ATPase and its nitric oxide-mediated regulation in a transgenic mouse model of amyotrophic lateral sclerosis. J Neurosci 23: 43-51, 2003.
8. Frey D, Schneider C, Xu L, Borg J, Spooren W, and Caroni P. Early and selective loss of neuromuscular synapse subtypes with low sprouting competence in motoneuron disease. J Neurosci 20: 2534-2542, 2000.
9. Gurney ME, Pu H, Chiu AY, Dal Canto MC, Polchow CY, Alexander DD, Caliendo J, Hentati A, Kwon YW, Deng HX, Chen W, Zhai P, Sufit RL, and Siddique T. Motor neuron degeneration in mice that express a human Cu, Zn superoxide dismutase mutation. Science 264: 1772-1775, 1994.
10. Hand CK and Rouleau GA. Familial amyotrophic lateral sclerosis. Muscle Nerve 25: 135-159, 2002.
11. Heath PR and Shaw PJ. Update on the glutamatergic neurotransmitter system and the role of excitotoxicity in amyotrophi lateral sclerosis. Muscle Nerve 26: 438-458, 2002.
12. Kaal EC, Vlug AS, Versleijen MW, Kuilman M, Joosten EA, and Bar PR. Chronic mitochondrial inhibition induces selective motoneuron death in vitro: a new model for amyotrophic lateral sclerosis. J Neurochem 74: 1158-1165, 2000.
13. Kong J and Xu Z. Massive mitochondrial degeneration in motor neurons triggers the onset of amyotrophic lateral sclerosis in mice expressing a mutant SOD1. J Neurosci 18: 3241-3250, 1998.
14. Kuo J, Fu R, Siddique T, and Heckman CJ. Persistent inward currents SOD1 transgenic mouse spinal cultures. Soc Neurosci Abstr 789.7, 2002.
15. Lancelot E, Lecanu L, Revaud ML, Boulu RG, Plotkine M, and Callebert J. Glutamate induces hydroxyl radical formation in vivo via activation of nitric oxide synthase in Sprague-Dawley rats. Neurosci Lett 242: 131-134, 1998.
16. Lee RH and Heckman CJ. Essential role of a fast persistent inward current in action potential initiation and control of rhythmic firing. J Neurophysiol 85: 472-475, 2001.
17. Li M, Ona VO, Guegan C, Chen M, Jackson-Lewis V, Andrews LJ, Olszewski AJ, Stieg PE, Lee JP, Przedborski S, and Friedlander RM. Functional role of caspase-1 and caspase-3 in an ALS transgenic mouse model. Science 288: 335-339, 2000.
18. Mogyoros I, Kiernan MC, Burke D, and Bostock H. Strength-duration properties of sensory and motor axons in amyotrophic lateral sclerosis. Brain 121: 851-859, 1998.
19. Palecek J, Lips MB, and Keller BU. Calcium dynamics and buffering in motoneurones of the mouse spinal cord. J Physiol 520: 485-502, 1999.
20. Powers RK and Binder MD. Input-output functions of mammalian motoneurons. Rev Physiol Biochem Pharmacol 143: 137-263, 2001.
21. Reaume AG, Elliott JL, Hoffman EK, Kowall NW, Ferrante RJ, Siwek DF, Wilcox HM, Flood DG, Beal MF, Brown RH Jr, Scott RW, and Snider WD. Motor neurons in Cu/Zn superoxide dismutase-deficient mice develop normally but exhibit enhanced cell death after axonal injury. Nat Genet 13: 43-47, 1996.
22. Rekling JC, Funk GD, Bayliss DA, Dong XW, and Feldman JL. Synaptic control of motoneuronal excitability. Physiol Rev 80: 767-852, 2000.
23. Rosen DR, Siddique T, Patterson D, Figlewicz DA, Sapp P, Hentati A, Donaldson D, Goto J, O'Regan JP, Deng HX, Rahmani Z, Krizus A, McKenna-Yasek D, Cayabyab A, Gaston SM, Berger R, Tanzi RE, Halperin JJ, Herzfeldt B, Van den Bergh R, Hung WY, Bird T, Deng G, Mulder DW, Smyth C, Laing NG, Soriano E, Pericak-Vance MA, Haines J, Rouleau GA, Gusella JS, Horvitz HR, and Brown RH. Mutations in Cu/Zn superoxide dismutase gene are associated with familial amyotrophic lateral sclerosis. Nature 362: 59-62, 1993.
24. Siddique T and Deng HX. Genetics of amyotrophic lateral sclerosis. Hum Mol Genet 5: 1465-1470, 1996.
25. Tu P-H, Raju P, Robinson KA, Gurney ME, Trojanowski JQ, and Lee VM-Y. Transgenic mice carrying a human mutant superoxide dismutase transgene develop neural cytoskeletal pathology resembling human amyotrophic sclerosis lesions. Proc Natl Acad Sci USA 93: 3155-3160, 1996.
26. Urbani A and Belluzzi O. Riluzole inhibits the persistent sodium current in mammalian CNS neurons. Eur J Neurosci 12: 3567-3574, 2000.
27. Vanselow BK and Keller BU. Calcium dynamics and buffering in oculomotor neurones from mouse that are particularly resistant during amyotrophic lateral sclerosis (ALS)-related motoneurone disease. J Physiol 525: 433-445, 2000.
28. Wong PC, Cai H, Borchelt DR, and Price DL. Genetically engineered mouse models of neurodegenerative diseases. Nat Neurosci 5: 633-639, 2002.
29. Zanette G, Tamburin S, Manganotti P, Refatti N, Forgione A, and Rizzuto N. Different mechanisms contribute to motor cortex hyperexcitability in amyotrophic lateral sclerosis. Clin Neurophysiol 113: 1688-1697, 2002.
30. Zona C, Ferri A, Gabbianelli R, Mercuri NB, Bernardi G, Rotilio G, and Carri MT. Voltage-activated sodium currents in a cell line expressing a Cu, Zn superoxide dismutase typical of familial ALS. Neuroreport 9: 3515-3518, 1998.