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337932-29-3Cc1ccc(C(=O)c2cncc(Br)c2)cc1AJZRSLVRFSCCIL-UHFFFAOYSA-N337932-29-3CHEMBL1413642안전 정보는 참고용이며, 공급업체의 물질안전보건자료(SDS), 관련 법규 및 전문가 평가를 대체하지 않습니다.
개요The 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.
용도Cuspin 1 is used in cuspin-1 characterization and regulation of SMN protein via Ras signaling in spinal muscular atropy.
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