Electrophysiological properties of motor neurons in a mouse model of severe spinal muscular atrophy: In Vitro versus in Vivo development

PLoS ONE

Volumn 5, Issue 7, 2010, Pages

  Zhang, Hongmei ^a   Robinson, Natallia ^a   Wu, Chiayen ^b   Wang, Wenlan ^b   Harrington, Melissa A ^a  

^a DELAWARE STATE UNIVERSITY   (United States)

^b NEMOURS ALFRED I DUPONT HOSPITAL FOR CHILDREN   (United States)

Author keywords

[No Author keywords available]

Indexed keywords

4 AMINOBUTYRIC ACID; SEROTONIN; SURVIVAL MOTOR NEURON PROTEIN 1;

ANIMAL CELL; ANIMAL EXPERIMENT; ANIMAL MODEL; ANIMAL TISSUE; ARTICLE; CELL SURVIVAL; CONTROLLED STUDY; DISEASE SEVERITY; ELECTRODE; ELECTROPHYSIOLOGY; EMBRYO; EMBRYO CULTURE; IN VITRO STUDY; IN VIVO STUDY; INTRACELLULAR RECORDING; LUMBAR SPINAL CORD; MOTONEURON; MOUSE; NERVE CELL MEMBRANE POTENTIAL; NONHUMAN; PATCH CLAMP; PROTEIN DEPLETION; PROTEIN EXPRESSION; SPINAL CORD VENTRAL HORN; SPINAL MUSCULAR ATROPHY; SYNAPTIC POTENTIAL; WHOLE CELL; ANIMAL; CELL CULTURE; DISEASE MODEL; METABOLISM; PATHOLOGY; PATHOPHYSIOLOGY; PHYSIOLOGY; SPINAL CORD;

ANIMALS; CELLS, CULTURED; DISEASE MODELS, ANIMAL; ELECTROPHYSIOLOGY; MICE; MOTOR NEURONS; MUSCULAR ATROPHY, SPINAL; PATCH-CLAMP TECHNIQUES; SPINAL CORD;

EID: 77955349353     PISSN: None     EISSN: 19326203     Source Type: Journal    
DOI: 10.1371/journal.pone.0011696     Document Type: Article

References (26)

1. Crawford TO, Pardo CA (1996) The neurobiology of childhood spinal muscular atrophy. Neurobiology of Disease 3: 97-110.
2. Iannaccone ST, Smith SA, Simard LR (2004) Spinal muscular atrophy. Current Neurology and Neuroscience Reports 4: 74-80.
3. Swash M, Desai J (2000) Motor neuron disease: Classification and nomenclature. Amyotroph Lateral Sclerosis and Other Motor Neuron Disorders 1: 105-112.
4. Talbot K, Davies KE (2001) Spinal muscular atrophy. Semin Neurol 21: 198-197.
5. Lefebvre S, Burglen L, Reboulett S, Clermont O, Burlet P, et al. (1995) Identification and characterization of a spinal muscular atrophy-determining gene. Cell 80: 155-165.
6. Monani UR (2005) Spinal muscular atrophy: a deficiency in a ubiquitous protein; a motor neuron-specific disease. Neuron 48: 885-896.
7. Lorson CL, Strasswimmer J, Yao JM, Baleja JD, Hahnen E, et al. (1999) SMN oligomerization defect correlates with spinal muscular atrophy severity. Nature Genetics 19: 63-66.
8. Monani UR, Lorson CL, Parsons DW, Prior TW, Androphy EJ, et al. (1999) A single nucleotide difference that alters splicing patterns distinguishes the SMA gene SMN1 from the copy gene SMN2. Human Molecular Genetics 8: 1177-1183.
9. Lorson CL, Androphy EJ (2000) An exonic enhancer is required for inclusion of essential exon in the SMA determining gene SMN. Human Molecular Genetics 9: 259-265.
10. Burghes AH, Beattie CE (2009) Spinal muscular atrophy: why do low levels of survival motor neuron protein make motor neurons sick? Nature Reviews Neuroscience 10: 597-609.
11. Monani UR, Sendtner M, Coovert DD, Parsons DW, Andreassi C, et al. (2000) The human centromeric survival motor neuron gene (SMN2) rescues embryonic lethality in Smn(-/-) mice, and results in a mouse with spinal muscular atrophy. Human Molecular Genetics 9: 333-339.
12. Rossoll W, Jablonka S, Andreassi C, Kroning AK, Karle K, et al. (2003) Smn the spinal muscular atrophy determining gene product, modulates axon growth and localization of B-actin mRNA in growth cones of motor neurons. Journal of Cell Biology 163: 801-812.
13. Ting CH, Lin CW, Wen SL, Hsieh-Li HM, Li H (2007) Stat5 constituitive activation rescues defects in spinal muscular atrophy. Human Molecular Genetics 16: 499-514.
14. Jablonka S, Beck M, Lechner B, Dorothea, Mayer C, Sendtner M (2007) Defective Ca2+ channel clustering in axon terminals disturbs excitability in motoneurons in spinal muscular atrophy. Journal of Cell Biology 179: 139-149.
15. McGovern VL, Gavrilina TO, Beattie CE, Burghes AH (2008) Embryonic motor axon development in the severe SMA mouse. Human Molecular Genetics 15: 2900-2909.
16. Kong L, Wang X, Choe DW, Polley M, Burnett BG, et al. (2009) Impaired synaptic vesicle release and immaturity of neuromuscular junctions in spinal muscular atrophy mice. J Neurosci 29: 842-851.
17. Schrank B, Gotz R, Gunnersen JM, Ure JM, Toyka KV, et al. (1997) Inactivation of the survival motor neuron gene, a candidate gene for human spinal muscular atrophy, leads to massive cell death in early mouse embryos. Proceedings of National Academy of Sciences, USA 94: 9920-9925.
18. Bendat JS, Piersol AG (2000) Random Data. Analysis and Measurement Procedures, 3rd ed., New York: John Wiley & Sons, Inc.
19. Smith S (2002) Digital Signal Processing: A Practical Guide for Engineers and Scientists Burlington, MA: Elsevier Science.
20. Quiroga RQ, Nadasdy Z, Ben-Shaul Y (2004) Unsupervised Spike Detection and Sorting with Wavelets and Superparamagnetic Clustering. Neural Computation 16: 1661-1687.
21. Zhang H, Robinson N, Gomez-Curet I, Wang W, Harrington M (2009) Neuronal and network activity in networks of cultured spinal motor neurons. NeuroReport 20: 854-859.
22. Swoboda KJ, Prior TW, Scott CB, McNaught TP, Wride MC, et al. (2005) Natural history of denervation in SMA: relation to age, SMN2 copy number, and function. Ann Neurol 57: 704-712.
23. Jablonka S, Wiese S, Sendtner M (2004) Axonal defects in mouse models of motoneuron disease. J Neurobiol 58: 272-286.
24. Kariya S, Park GH, Maeno-Hikichi Y, Leykekhman O, Lutz C, et al. (2008) Reduced SMN protein impairs maturation of the neuromuscular junctions in mouse models of spinal muscular atrophy. Hum Mol Genet 17: 2552-2569.
25. Noakes PG, Phillips WD, Hanley TA, Sanes JR, Merlie JP (1993) 43K protein and acetylcholine receptors colocalize during the initial stages of neuromuscular synapse formation in vivo. Dev Biol 155: 275-280.
26. Alpaydin E (2004) Introduction to Machine Learning. Cambridge, MA: The MIT Press.