Cuspin-1

The Survival of Motor Neurons (SMN) protein participates in RNA splicing. Decreases in SMN, typically a consequence of defects in the smn1 gene, result in the death of motor neurons and lead to the neurodegenerative disease, spinal muscular atrophy (SMA). Cuspin-1 is a small molecule upregulator of SMN that has been shown in vitro to increase levels of SMN in SMA patient fibroblasts by 50% at 18 µM. Its mechanism of action is thought to involve increased phosphorylation of ERK to initiate Ras-Raf-MEK signaling, which results in an increased rate of SMN translation.Formal Name: (5-bromo-3-pyridinyl)(4-methylphenyl)-methanone. CAS Number: 337932-29-3. Synonyms: Chemical Upregulator of SMN Protein-1. Molecular Formula: C13H10BrNO. Formula Weight: 276.1. Purity: >98%. Formulation: (Request formulation change), A crystalline solid. Solubility: DMF: 25 mg/mL, DMF:PBS(pH 7.2)(1:1): 0.5 mg/ml, DMSO: 16 mg/mL, Ethanol: 1 mg/mL. lambdamax: 211, 270 nm. SMILES: CC1=CC=C(C(C2=CC(Br)=CN=C2)=O)C=C1. InChi Code: InChI=1S/C13H10BrNO/c1-9-2-4-10(5-3-9)13(16)11-6-12(14)8-15-7-11/h2-8H,1H3. InChi Key: AJZRSLVRFSCCIL-UHFFFAOYSA-N.