ERCA: Sarcoplasmic reticulum Ca2+ ATPase; SMA: Spinal muscular atrophy; SMN: Survival motor neuron; SV2: Synaptic vesicle protein two; TA: Tibialis anterior; ZEB: Zinc-finger E box-binding protein. Competing interests The authors declared that they’ve no competing interests. Authors’ contributions JGB and RK conceived and developed the project. JGB performed and analyzed a lot of the experiments and was assisted by KS for Figures 1 and three, and by YDR for panels A and C of Figure two. LMM performed experiments and analyzed the data in panels E-H of Figure 2. JMR supervised KS and helped analyze the information in Figure 1. JGB wrote the paper and RK revised and edited it. All authors study and approved the final manuscript. Acknowledgements We thank Marc-Olivier Deguise for outstanding technical assistance. This project was funded by grants from the Canadian Institutes of Health Study (CIHR) plus the Muscular Dystrophy Association (USA) to RK. JGB is a recipient of a Frederick Banting and Charles Finest CIHR Doctoral Research Award, LMM can be a recipient of a A number of Sclerosis Society of Canada Postdoctoral Fellowship, and RK can be a recipient of a University Overall health Analysis Chair from the University of Ottawa.C-Phycocyanin manufacturer Author details 1 Ottawa Hospital Research Institute, Regenerative Medicine Program, 501 Smyth Road, Ottawa, ON K1H 8L6, Canada. 2Department of Cellular and Molecular Medicine, University of Ottawa, Ottawa, ON K1H 8M5, Canada. 3 Division of Medicine, University of Ottawa, Ottawa, ON K1H 8M5, Canada. Received: 18 July 2013 Accepted: 26 September 2013 Published: 11 October9. References 1. Prior TW, Snyder PJ, Rink BD, Pearl DK, Pyatt RE, Mihal DC, Conlan T, Schmalz B, Montgomery L, Ziegler K, Noonan C, Hashimoto S, Garner S: Newborn and carrier screening for spinal muscular atrophy. Am J Med Genet A 2010, 152A(7):1608616. two. Pearn J: Incidence, prevalence, and gene frequency studies of chronic childhood spinal muscular atrophy. J Med Genet 1978, 15(6):40913. 3. Ogino S, Leonard DG, Rennert H, Ewens WJ, Wilson RB: Genetic risk assessment in carrier testing for spinal muscular atrophy. Am J Med Genet 2002, 110(4):30107. 4. Boyer JG, Bowerman M, Kothary R: The numerous faces of SMN: deciphering the function crucial to spinal muscular atrophy pathogenesis.Ionomycin Apoptosis,Neuronal Signaling,Epigenetics,TGF-beta/Smad,Anti-infection,Membrane Transporter/Ion Channel Future Neurol 2010, five(6):87390.PMID:23489613 five. Lefebvre S, B glen L, Reboullet S, Clermont O, Burlet P, Viollet L, Benichou B, Cruaud C, Millasseau P, Zeviani M, Le Paslier D, Fr al J, Cohen D, Weissenbach J, Munnich A, Melki J: Identification and characterization of a spinal muscular atrophy-determining gene. Cell 1995, 80(1):15565. 6. Brzustowicz LM, Merette C, Kleyn PW, Lehner T, Castilla LH, Penchaszadeh GK, Das K, Munsat TL, Ott J, Gilliam TC: Assessment of nonallelic genetic heterogeneity of chronic (sort II and III) spinal muscular atrophy. Hum Hered 1993, 43(6):38087. 7. Melki J, Abdelhak S, Sheth P, Bachelot MF, Burlet P, Marcadet A, Aicardi J, Barois A, Carriere JP, Fardeau M, Fontan D, Ponsot G, Billette T, Angelini C, Barbosa C, Ferriere G, Lanzi G, Ottolini A, Babron MC, Cohen D, Hanauer A, Clerget-Darpoux F, Lathrop M, Munnich A, Frezal J: Gene for chronic proximal spinal muscular atrophies maps to chromosome 5q. Nature 1990, 344(6268):76768. 8. Rochette CF, Gilbert N, Simard LR: SMN gene duplication as well as the emergence in the SMN2 gene occurred in distinct hominids: SMN2 is exceptional to Homo sapiens. Hum Genet 2001, 108(3):25566. 27.28.Schrank B, Gotz R, Gu.