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FSMA Funded Articles in 2006
 
1. Singh NN, Singh RN, Androphy EJ.
Modulating role of RNA structure in alternative splicing of a critical exon in the spinal muscular atrophy genes.
Nucleic Acids Research.  December 14, 2006.

2. Butchbach ME, Edwards JD, Schussler KR, Burghes AH.
A novel method for oral delivery of drug compounds to the neonatal SMNDelta7 mouse model of spinal muscular atrophy.
Journal of Neuroscience Methods. December 8, 2006.

3. Carrel TL, McWhorter ML, Workman E, Zhang H, Wolstencroft EC, Lorson C, Bassell GJ, Burghes AH, Beattie CE.
Survival motor neuron function in motor axons is independent of functions required for small nuclear ribonucleoprotein biogenesis.
Journal of Neuroscience. October 25, 2006.

4. Singh RN.
Association of severity of spinal muscular atrophy with the loss of NAIP gene.
Neurology India. September 2006.

5. Zhang H, Xing L, Rossoll W, Wichterle H, Singer RH, Bassell GJ.
Multiprotein complexes of the survival of motor neuron protein SMN with Gemins traffic to neuronal processes and growth cones of motor neurons.
Journal of Neuroscience. August 16, 2006.

6. Salah-Mohellibi N, Millet G, Andre-Schmutz I, Desforges B, Olaso R, Roblot N, Courageot S, Bensimon G, Cavazzana-Calvo M, Melki J.
Bone marrow transplantation attenuates the myopathic phenotype of a muscular mouse model of spinal muscular atrophy.
Stem Cells. December 2006.

8. Hahnen E, Eyupoglu IY, Brichta L, Haastert K, Trankle C, Siebzehnrubl FA, Riessland M, Holker I, Claus P, Romstock J, Buslei R, Wirth B, Blumcke I.
In vitro and ex vivo evaluation of second-generation histone deacetylase inhibitors for the treatment of spinal muscular atrophy.
Journal of Neurochemistry. July 2006. 

9. Krosschell KJ, Maczulski JA, Crawford TO, Scott C, Swoboda KJ.
A modified Hammersmith functional motor scale for use in multi-center research on spinal muscular atrophy.
Neuromuscular Disorders. July 2006. 

10. Riessland M, Brichta L, Hahnen E, Wirth B.
The benzamide M344, a novel histone deacetylase inhibitor, significantly increases SMN2 RNA/protein levels in spinal muscular atrophy cells.
Human Genetics. August 2006. 

11. Doran B, Gherbesi N, Hendricks G, Flavell RA, Davis RJ, Gangwani L.
Deficiency of the zinc finger protein ZPR1 causes neurodegeneration.
Proceedings of the National Academy of Science U S A. May 2006. 

12. Brichta L, Holker I, Haug K, Klockgether T, Wirth B.
In vivo activation of SMN in spinal muscular atrophy carriers and patients treated with valproate.
Annals of Neurology June 2006.

13. Baughan T, Shababi M, Coady TH, Dickson AM, Tullis GE, Lorson CL.  
Stimulating full-length SMN2 expression by delivering bifunctional RNAs via a viral vector.
Molecular Therapeutics. July 2006. 

14. Singh NK, Singh NN, Androphy EJ, Singh RN.
Splicing of a critical exon of human Survival Motor Neuron is regulated by a unique silencer element located in the last intron.
Molecular and Cellular Biology.  February 2006. 

15. Deshpande DM, Kim YS, Martinez T, Carmen J, Dike S, Shats I, Rubin LL, Drummond J, Krishnan C, Hoke A, Maragakis N, Shefner J, Rothstein JD, Kerr DA.
Recovery from paralysis in adult rats using embryonic stem cells.
Annals of Neurology.  July 2006.

16. Gangwani L.
Deficiency of the zinc finger protein ZPR1 causes defects in transcription and cell cycle progression.
Journal of Biological Chemistry. December 29, 2006.

17. Markus, M. A., Heinrich, B., Raitskin, O., Adams, D. J., Mangs, H., Goy, C., Ladomery, M., Sperling, R., Stamm, S., and Morris, B. J. WT1 interacts with the splicing protein RBM4 and regulates its ability to modulate alternative splicing in vivo. Experimental Cell Research. 2006.

18.Wirth B, Brichta L, Schrank B, Lochmuller H, Blick S, Baasner A,  Heller R.    
Mildly affected patients with spinal muscular atrophy are partially protected by an increased SMN2 copy number.
Human Genetics. 2006 May

 

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