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37

抑制剂 & 化合物

6

天然产物

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Cat. No.	Product Name	Target	Signaling Pathways
T39924	NF-κΒ activator 2	NF-κB	NF-κB
	NF-κΒ activator 2 是口服有效的 NF-ҡB 激活剂，EC50为 1.58 μM。它通过增加 NF-κB 的表达和激活来诱导 SOD2。它具有用于研究肌萎缩性侧索硬化症的价值。
T13553	Arimoclomol 阿瑞洛莫,BRX-220 free base	HSP	Cytoskeletal Signaling; Metabolism
	Arimoclomol (BRX-220 free base) 是热休克蛋白 (HSP) 的共诱导剂，可用于治疗肌萎缩侧索硬化症的研究。
T0349	Riluzole PK 26124,RP-54274,利鲁唑	GABA Receptor; Sodium Channel; NMDAR	Membrane transporter/Ion channel; Neuroscience
	Riluzole (RP-54274) 是一种谷氨酸拮抗剂，用作抗惊厥药并延长肌萎缩侧索硬化症患者的生存期。它还抑制 GABA 摄取，IC50值为 43 μM。
T60507	Utreloxastat EPI 857,PTC857	Lipoxygenase	Metabolism
	Utreloxastat (PTC857) 是一种新型 15-脂氧合酶抑制剂，可用于研究肌萎缩侧索硬化症。
T60120	SOD1-Derlin-1 inhibitor 56-59	Others	Others
	SOD1-Derlin-1 inhibitor 56-59是SOD1-Dderin-1相互作用的抑制剂。SOD1-Derlin-1 inhibitor 56-59在肌萎缩侧索硬化症（ALS）研究中具有潜力。
T21995	XAP044	GluR	Neuroscience
	XAP044 是高效的选择性mGlu7拮抗剂。代谢型谷氨酸受体亚型 7 是哺乳动物 CNS 中神经传递的重要突触前调节剂。它在啮齿动物行为测试中表现出较好的大脑暴露、广谱抗应激、抗抑郁以及抗焦虑作用。
T60119	SOD1-Derlin-1 inhibitor-1 SOD1-Derlin-1 Inhibitor 56-20	Others	Others
	SOD1-Derlin-1 inhibitor-1 (SOD1-Derlin-1 Inhibitor 56-20) (compound 56-20) 是 SOD1-Derlin-1相互作用的抑制剂。SOD1-Derlin-1 inhibitor-1 抑制 SOD1G93A-Derlin-1 复合物，IC50值为 7.11 μM。SOD1-Derlin-1 inhibitor-1 可用于肌萎缩性侧索硬化症的研究。
T60108	PDGFR Tyrosine Kinase Inhibitor III PDGF Receptor Tyrosine Kinase Inhibitor III	PDGFR	Angiogenesis; Tyrosine Kinase/Adaptors
	PDGFR Tyrosine Kinase Inhibitor III (PDGF Receptor Tyrosine Kinase Inhibitor III) (PDGF Receptor Tyrosine Kinase Inhibitor III)是一种多激酶抑制剂，可抑制 PDGFR、EGFR、FGFR、PKA 和 PKC 等。PDGFR Tyrosine Kinase Inhibitor III 可用于肌萎缩侧索硬化症的研究。
T77484	Atibuclimab	TNF	Apoptosis
	Atibuclimab 是一种靶向 CD14 的嵌合单克隆抗体，由小鼠可变区和人类 IgG4 Fc 区构成。Atibuclimab 可用于治疗肌萎缩侧索硬化症。Atibuclimab 可减少 LPS 诱导的病症，并对 LPS 诱导的促炎细胞因子释放有抑制作用，可延迟抗炎细胞因子可溶性 TNF 受体 I 型的释放，
T14390L	(2R,3S)-Azelaprag	Apelin receptor	GPCR/G Protein
	(2R,3S)-N-(4-(2,6-dimethoxyphenyl)-5-(5-methylpyridin-3-yl)-4H-1,2,4-triazol-3-yl)-3-(5-methylpyrimidin-2-yl)butane-2-sulfonamide 是一种Apelin 受体激动剂，对Apelin 受体的EC50为0.012 µM。(2R,3S)-N-(4-(2,6-dimethoxyphenyl)-5-(5-methylpyridin-3-yl)-4H-1,2,4-triazol-3-yl)-3-(5-methylpyrimidin-2-yl)butane-2-sulfonamide 可用于治疗心力衰竭、动脉粥样硬化、肥胖、糖尿病、肌萎缩侧索硬化、冠心病、高血压、中风和心肌梗塞等疾病。
T35689	MTP 131 acetate	Others	Others
	MTP 131 acetate 是一种小的线粒体靶向四肽。
T31978	GM-608 GM-606,GM-607,GM-604,GM-603,GM-605,GM-602
	GM-604(alirinetide) is a potential treatment for amyotrophic lateral sclerosis and ischemic stroke.
T24814	Sortin2 Sortin-2,Sortin 2
	Sortin2 is a sorting inhibitor that acts upstream from the morphological marker of lateral root primordium formation, the mitotic activity.
T70892	K812
	K812 is an ASK1-specific inhibitor discovered to prolong survival in a mouse model of amyotrophic lateral sclerosis.
T25852	Naxillin
	Naxillin is the first non-auxin-like molecule that promotes root branching which acts as a specific lateral root development modulator.
T35135	WN1316 WN 1316,WN-1316
	WN1316 is a unique neuroprotectant against oxidative injury, being a highly promising remedy for the treatment of amyotrophic lateral sclerosis (ALS).
T11214	EPI-589	Others	Others
	EPI-589, a quinone derivative and oxidoreductase enzyme inhibitor, is considered safe and well tolerated, showing promise for the treatment of amyotrophic lateral sclerosis (ALS).
T70893	K811
	K811 is an ASK1-specific inhibitor that prolongs survival in a mouse model of amyotrophic lateral sclerosis. K811 efficiently prevented cell proliferation in cell lines with high ASK1 expression and in HER2-overexpressing GC cells. Treatment with K811 reduced sizes of xenograft tumors by downregulating proliferation markers.
T82704	CMB-087229
	CMB-087229为一种针对突变SOD1蛋白的聚集阻断剂，具有IC50值为67 nM，主要用于肌萎缩侧索硬化症的研究。
T72959	NUCC-0000323
	NUCC-0000323 是一种针对超氧化物歧化酶 1 (SOD1) 的有效抑制剂，能够抑制 SOD1 的表达。该化合物在肌萎缩侧索硬化症 (ALS) 研究中有潜在应用。
T39498	Tofersen BIIB067,ISIS-SOD1Rx
	Tofersen (BIIB067) is an antisense oligonucleotide utilized to inhibit the synthesis of superoxide dismutase 1 (SOD1) protein by facilitating the degradation of SOD1 mRNA via the RNase H-dependent mechanism. This compound holds potential for amyotrophic lateral sclerosis (ALS) research.
T81372	Proteasome-activating peptide 1
	Proteasome-activating peptide 1 是一种肽，其作用是提升胰凝乳蛋白酶样蛋白酶体（proteasome）的催化活性，有效增强体外及细胞培养环境下的蛋白质降解效率。此外，该肽能阻止肌萎缩侧索硬化症（Amyotrophic Lateral Sclerosis, ALS）细胞模型中蛋白质的过度聚集。
T81580	Ozanezumab GSK1223249
	Ozanezumab (GSK1223249) 是一种anti-Nogo-A 单克隆抗体，用于肌萎缩侧索硬化症 (ALS) 及多发性硬化症研究。
T61128	c-ABL-IN-2
	C-ABL-IN-2 is a highly effective inhibitor of the c-Abl protein, which plays a significant role in the development of several diseases, including cancer. This compound exhibits promise for investigating neurodegenerative disorders such as amyotrophic lateral sclerosis (ALS) and Parkinson's disease (PD), as well as cancer. (Source: WO2020260871A1, compound 25) [1].
T61546	c-ABL-IN-3
	c-ABL-IN-3, a highly effective c-Abl inhibitor, exhibits promising potential for studying neurodegenerative diseases such as amyotrophic lateral sclerosis (ALS) and Parkinson's disease (PD), as well as cancer [1]. The activation of c-Abl has been strongly implicated in a range of diseases, notably cancer [1]. It offers valuable opportunities for research in these fields [1].
T82560	di-Ellipticine-RIBOTAC	PROTACs	PROTAC
	Di-Ellipticine-RIBOTAC是一款双功能小分子，在体外和体内肌萎缩侧索硬化（ALS）模型中能够减少c9ALS/FTD r(G4C2)的重复扩增。
TP1542	26Rfa, Hypothalamic Peptide, human
	This human neuropeptide of the RFamide peptide family with orexigenic activity may be also involved in the control of feeding behavior. The 26RFa gene is exclusively expressed in the ventromedial hypothalamic nucleus and in the lateral hypothalamic area.
T73117	ROCK2-IN-5
	ROCK2-IN-5是一种复合型化合物，融合了Rho激酶抑制剂fasudil以及NRF2诱导剂咖啡酸和阿魏酸的结构元素。该化合物展示了优秀的多靶点作用特性和较高的耐受性。ROCK2-IN-5在针对SOD1基因变异型ALS研究中具备应用潜力。
T78603	Sotuletinib dihydrochloride BLZ945 dihydrochloride	c-Fms	Tyrosine Kinase/Adaptors
	Sotuletinib (BLZ945) dihydrochloride 是一种CSF1-R特异性抑制剂，具有口服活性和血脑屏障透过性 (IC50=1 nM)。它能诱导肿瘤细胞凋亡，并在小鼠模型中有效抑制肿瘤生长，适用于癌症和肌萎缩侧索硬化症 (ALS) 研究。
T37608	Riluzole-13C,15N2 Riluzole-13C,15N2
	Riluzole-13C,15N2 is intended for use as an internal standard for the quantification of riluzole by GC- or LC-MS. Riluzole is a benzothiazole derivative with anti-excitotoxic effects that acts by blocking the presynaptic release of glutamate, indirectly antagonizing glutamate receptors, and inactivating neuronal voltage-gated Na+ channels (ED50 = 2.3 μM). Riluzole suppresses glutamate-induced seizures in rats at an ED50 value of 3.2 mg/kg and displays neuroprotective effects in hypoxic animals a...
T76243	Proteasome-activating peptide 1 TFA
	Proteasome-activating peptide 1 TFA 是一种肽类蛋白酶体激活剂，能显著增强胰凝乳蛋白酶样蛋白酶体的催化活性，进而提升体外及培养环境中的蛋白质水解效率。此外，Proteasome-activating peptide 1 TFA 还能有效阻止肌萎缩侧索硬化症细胞模型内的蛋白质聚集现象。
T60966	UCPH-102
	UCPH-102 可用于研究阿尔茨海默病、肌萎缩性侧索硬化症、慢性疼痛和强迫症等，具有良好的血脑通透性。UCPH-102 是高度选择性的 EAAT1抑制剂，IC50值为0.43 μM。UCPH-102 还对 T-ALL 细胞表现出特异性的抗增殖作用。
T60953	BSc5367
	BSc5367 是Nek1激酶结构域的有效抑制剂，IC50值为 11.5 nM。Nek1 是一种NIMA 相关的蛋白激酶，在细胞周期调节、DNA 修复和微管调节中起着关键作用。Nek1 功能障碍在肌萎缩侧索硬化症 (ALS)、多囊肾病 (PKD) 和几种类型的放疗耐药性癌症中发挥关键作用。BSc5367 为更好的理解上述疾病提供了有用工具。
T36499	CuATSM
	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 ...
TMIH-0552	Tauroursodeoxycholic Acid-d4 Sodium Salt
	Tauroursodeoxycholic Acid-d4 Sodium Salt 是 Tauroursodeoxycholic Acid Sodium Salt 的氘代化合物。Tauroursodeoxycholic Acid Sodium Salt 的 CAS 号为 14605-22-2。Tauroursodeoxycholate，又称 ursodoxicoltaurine，是一种高度亲水性的三级胆汁酸，在人体内以低浓度产生。Tauroursodeoxycholate 是 ursodeoxycholic acid 更亲水的形式，而 ursodeoxycholic acid 是人类体内自然产生的更丰富的胆汁酸。Tauroursodeoxycholate 正在研究用于几种疾病，如原发性胆汁性肝硬化(PBC)、胰岛素抵抗、淀粉样变性、囊性纤维化、胆汁淤滞和肌萎缩性侧索硬化症。
T38218	1-Stearoyl-2-docosahexaenoyl-sn-glycero-3-PC
	1-Stearoyl-2-docosahexaenoyl-sn-glycero-3-PC 是一种在生物膜中的磷脂，在 sn-1 和 sn-2 位置分别含有硬脂酸和二十二碳六烯酸。它已被用于研究脂膜的组织和动力学。在超氧化物歧化酶 1 突变体转基因小鼠肌萎缩性脊髓侧索硬化症（ALS）模型中1-Stearoyl-2-docosahexaenoyl-sn-glycero-3-PC 的水平在疾病晚期会在 L5 前角下降。1-Stearoyl-2-docosahexaenoyl-sn-glycero-3-PC 可用于研究生物膜。
T38286	C26 Sphingomyelin (d18:1/26:0) C26 Sphingomyelin (d18:1/26:0)
	C26 Sphingomyelin is a sphingolipid that has been found in serum and cell or brain extracts.1,2,3C26 sphingomyelin levels are increased in the serum of patients with hemophagocytic lymphohistiocytosis, an inflammatory condition characterized by excessive immune activation.3C26 sphingomyelin has been used a component of monolayers to study the influence of sphingomyelin acyl chain length on sphingomyelin-sterol interactions.4[Matreya, LLC.] 1.Wang, J.-R., Zhang, H., Yau, L.D., et al.Improved sphi...

化合物

NF-κΒ activator 2

Cat.No: T39924

Target: NF-κB

Cat.No: T13553

Synonym: 阿瑞洛莫,BRX-220 free base

Target: HSP

Cat.No: T0349

Synonym: PK 26124,RP-54274,利鲁唑

Target: GABA Receptor, Sodium Channel, NMDAR

Cat.No: T60507

Synonym: EPI 857,PTC857

Target: Lipoxygenase

SOD1-Derlin-1 inhibitor 56-59

Cat.No: T60120

Target: Others

Cat.No: T21995

Target: GluR

SOD1-Derlin-1 inhibitor-1

Cat.No: T60119

Synonym: SOD1-Derlin-1 Inhibitor 56-20

Target: Others

PDGFR Tyrosine Kinase Inhibitor III

Cat.No: T60108

Synonym: PDGF Receptor Tyrosine Kinase Inhibitor III

Target: PDGFR

Cat.No: T77484

Target: TNF

(2R,3S)-Azelaprag

Cat.No: T14390L

Target: Apelin receptor

MTP 131 acetate

Cat.No: T35689

Target: Others

Cat.No: T31978

Synonym: GM-606,GM-607,GM-604,GM-603,GM-605,GM-602

Cat.No: T24814

Synonym: Sortin-2,Sortin 2

Cat.No: T70892

Cat.No: T25852

Cat.No: T35135

Synonym: WN 1316,WN-1316

Cat.No: T11214

Target: Others

Cat.No: T70893

Cat.No: T82704

Cat.No: T72959

Cat.No: T39498

Synonym: BIIB067,ISIS-SOD1Rx

Proteasome-activating peptide 1

Cat.No: T81372

Cat.No: T81580

Synonym: GSK1223249

Cat.No: T61128

Cat.No: T61546

di-Ellipticine-RIBOTAC

Cat.No: T82560

Target: PROTACs

26Rfa, Hypothalamic Peptide, human

Cat.No: TP1542

Cat.No: T73117

Sotuletinib dihydrochloride

Cat.No: T78603

Synonym: BLZ945 dihydrochloride

Target: c-Fms

Riluzole-13C,15N2

Cat.No: T37608

Synonym: Riluzole-13C,15N2

Proteasome-activating peptide 1 TFA

Cat.No: T76243

Cat.No: T60966

Cat.No: T60953

Cat.No: T36499

Tauroursodeoxycholic Acid-d4 Sodium Salt

Cat.No: TMIH-0552

1-Stearoyl-2-docosahexaenoyl-sn-glycero-3-PC

Cat.No: T38218

C26 Sphingomyelin (d18:1/26:0)

Cat.No: T38286

Synonym: C26 Sphingomyelin (d18:1/26:0)

Cat. No.	Product Name	Target	Signaling Pathways
T22911	β-N-methylamino-L-alanine hydrochloride L-BMAA hydrochloride	Others	Others
	β-N-methylamino-L-alanine hydrochloride (L-BMAA hydrochloride) 是由蓝细菌产生的一种神经毒素，既有导致肌萎缩性侧索硬化症 (ALS) 以及其他可能的神经退行性疾病可能性。
TMS2179	Zeatin 玉米素	Others	Others
	Zeatin 是一种来源于腺嘌呤的细胞分裂素。Zeatin 是在玉米属的未成熟玉米粒中发现的。它促进侧芽的生长，当喷洒在分生组织上时会刺激细胞分裂，从而产生更浓密的植物。
T2532	Tauroursodeoxycholate Taurolite,牛磺熊去氧胆酸,Tauroursodeoxycholic Acid,UR 906,Ursodeoxycholyltaurine,TUDCA	Apoptosis; ERK; P450; Caspase; Endogenous Metabolite	Apoptosis; MAPK; Metabolism; Proteases/Proteasome
	Tauroursodeoxycholate (UR 906)，又称 ursodoxicoltaurine，是一种高度亲水性的三级胆汁酸，在人体内以低浓度产生。Tauroursodeoxycholate 是 ursodeoxycholic acid 更亲水的形式，而 ursodeoxycholic acid 是人类体内自然产生的更丰富的胆汁酸。Tauroursodeoxycholate 正在研究用于几种疾病，如原发性胆汁性肝硬化(PBC)、胰岛素抵抗、淀粉样变性、囊性纤维化、胆汁淤滞和肌萎缩性侧索硬化症。
T37298	Spinacine	Endogenous Metabolite	Metabolism
	Spinacine 是人内源性代谢产物。
T82407	Feruloyl tyramine
	Feruloyl tyramine 为一酰胺类化合物，可从大麻籽乙醇提取物中分离。在小鼠侧脑室注射该化合物，可引发体温降低及运动协调障碍。
T75527	Chrexanthomycin C
	Chrexanthomycin C 是一种具有显著生物活性的海洋天然产物，具有口服活性。Chrexanthomycin C 对 DNA (G4C2)4 G4 具有结合亲和力，Kd 值为 2.8 mM。Chrexanthomycin C 可用于肌萎缩侧索硬化症 (ALS) 等神经退行性疾病的研究。

天然产物

β-N-methylamino-L-alanine hydrochloride

Cat.No: T22911

Synonym: L-BMAA hydrochloride

Target: Others

Cat.No: TMS2179

Synonym: 玉米素

Target: Others

Tauroursodeoxycholate

Cat.No: T2532

Synonym: Taurolite,牛磺熊去氧胆酸,Tauroursodeoxycholic Acid,UR 906,Ursodeoxycholyltaurine,TUDCA

Target: Apoptosis, ERK, P450, Caspase, Endogenous Metabolite

Cat.No: T37298

Target: Endogenous Metabolite

Feruloyl tyramine

Cat.No: T82407

Chrexanthomycin C

Cat.No: T75527

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