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- HIT ID:HIT100110890
- Cas No:337932-29-3
- 常用名:Cuspin-1
- InChI key:AJZRSLVRFSCCIL-UHFFFAOYSA-N
- 分子式:C13H10BrNO
- 分子量:276.133
- 氢键供体数 (HBD):0
- 氢键受体数 (HBA):3
- LogP:3.7289
- 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.
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