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Cuspin-1 (Chemical Upregulator of SMN Protein-1) is an upregulator of the Survival of Motor Neuron protein (SMN), necessary for survival of motor neurons. SMN is decreased in the neurodegenerative disease Spinal Muscular Atrophy (SMA), an autosomal recessive disease caused by a genetic defect in the SMN1 gene. SMA is the second leading cause of neuromuscular disease, after Duchenne muscular dystrophy. Cuspin-1 increased SMN levels by 50-100% in SMA patient fibroblast cells accompanied by an increase in the phosphorylation of Erk, suggesting inbvolvement of the Ras-Raf-MEK cascade.
| Molecular Weight | 276.13 |
| Formula | C13H10BrNO |
| CAS Number | 337932-29-3 |
| Solubility (25°C) | DMSO: 5 mg/mL (warmed) |
| Storage |
Powder -20°C 3 years ; 4°C 2 years In solvent -80°C 6 months ; -20°C 1 month |
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