misfolding

Icerguastat (IFB-088) 是一种缺乏 α2-肾上腺素能活性的 Guanabenz 衍生物，是一种磷酸酶调节亚单位 PPP1R15A (R15A)的选择性抑制剂。它能够抑制 eIF2α 去磷酸化，从而延长保护反应。它具有抗朊病毒作用。

Sephin1 (IFB-088) 是 GADD34 (PPP1R15A) 的选择性抑制剂，这是一种应激诱导的蛋白磷酸酶 1 复合物调节亚基，可将 eIF2α 去磷酸化。

VAS 3947 是一种特异的 NADPH 氧化酶 (NOX) 抑制剂。VAS 3947具有强大的抗血小板作用, 通过 UPR 激活，主要由于蛋白质聚集和错误折叠，独立于抗 NOX 活性诱导细胞凋亡。

Quinacrine is a compound that is commonly used as an anti-protozoal agent. It inhibits voltage-dependent sodium channels (IC50: 3.3 μM) and suppresses aldehyde oxidase (IC50: 3.3 μM). Quinacrine prevents misfolding of prion protein (EC50: 0.3 μM). As an e

IFB-088 acetate 是一种苄基胍衍生物，可用于治疗与PPP1R15A 途径相关并与蛋白质错误折叠应激相关的疾病和癌症，如tau 病、突触核蛋白病、多谷氨酰胺和多丙氨酸疾病、白质营养不良、囊性纤维化、多发性硬化症、溶酶体储存障碍、淀粉样变性疾病、炎症、代谢障碍、心血管疾病、骨质疏松症、神经系统创伤等等。

Fe-TMPyP binds to the prion protein PrP and inhibits misfolding. Fe-TMPyP is also a peroxynitrite decomposition catalyst.

NPT200-11 是一种口服生物利用度和脑穿透性 ASYN 错误折叠和聚集抑制剂。 NPT200-11 可用于突触核蛋白病的潜在病理学研究，包括帕金森病 (PD)、路易体痴呆 (DLB) 和多系统萎缩 (MSA)。

Minzasolmin（DLX-313） 是一种 α-突触核蛋白错误折叠抑制剂，可用于研究帕金森疾病。

CFTRcorrector 14 (Compound SVQ26) 作为一种第三类囊性纤维化跨膜传导调节剂(CFTR)校正剂,具备在C1类校正剂VX-809存在下提高CFTR活性的能力,其EC50值为3.08 μM.此化合物能够调整由突变导致的CFTR蛋白错误折叠及功能障碍,适用于囊性纤维化的相关研究.

The metallo-protein Cu/Zn-superoxide dismutase (SOD1) is a ubiquitous enzyme responsible for scavenging superoxide radicals. Mutations in SOD1, which alter its metal binding capacity and can result in protein misfolding and aggregation, have been linked to familial amyotrophic lateral sclerosis (ALS). Cu-ATSM is an orally bioavailable, blood-brain barrier permeable complex that has traditionally been used in cellular imaging experiments to selectively label hypoxic tissue via its susceptibility to reduction by oxygen-depleted mitochondria. More recently, Cu-ATSM has been reported to improve locomotor function and survival in a transgenic ALS mouse model by delivering copper specifically to cells in the spinal cords of mice producing misfolded SOD1 proteins. Copper chaperone for SOD (CCS) is presumed to utilize the copper from Cu-ATSM to prevent misfolding of the SOD1 protein.