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Cat. No. | Product Name | Target | Signaling Pathways |
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T78573 |
Glucocerebrosidase
GCase,Glucosylceramidase |
Endogenous Metabolite | Metabolism |
Glucocerebrosidase (Glucosylceramidase; GBA) 是溶酶体内的酶,负责催化GC中的β-糖苷键裂解,从而生成葡萄糖和神经酰胺。 | |||
T60353 |
Glucocerebrosidase-IN-1
|
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Glucocerebrosidase-IN-1 (compound 11a) 是一种有效的选择性的GCase(葡糖脑苷脂酶)抑制剂,其IC50为 29.3 μM,Ki 为 18.5 μM。由于GCase 参与酶缺乏性病理疾病,Glucocerebrosidase-IN-1 可用于戈谢病 (GD) 和帕金森病 (PD) 的研究。 | |||
T78671 |
Glucocerebrosidase-IN-1 hydrochloride
|
Glucosidase | Metabolism |
Glucocerebrosidase-IN-1 (compound 11a) hydrochloride,是一种针对GCase的高效选择性抑制剂,展现出了29.3 μM的IC50和18.5 μM的Ki。该化合物适用于戈谢病(GD)与帕金森病(PD)相关研究。 | |||
T8986 |
ML198
|
Glucokinase | Metabolism |
ML198 是葡萄糖脑苷脂酶激活剂(EC50:0.4 μM)。它不会抑制该酶的作用,且能促进其易位至溶酶体,可用于研究戈谢病。 | |||
T0920 |
Ambroxol
氨溴索,氨溴索碱,Ambroxolum,NA-872 |
P450; Autophagy | Autophagy; Metabolism |
Ambroxol (NA-872) 是前药溴己辛的活性代谢产物,是一种分泌溶解剂,具有强效祛痰作用,用于治疗与粘液或过多粘液相关的呼吸道疾病。它可诱导肺组织细胞自噬,有用于帕金森氏病和神经性戈谢病的研究潜力。它是一种葡糖脑苷脂酶伴侣,可增加葡糖脑苷脂酶的活性。 | |||
T6234 |
Ambroxol hydrochloride
Mucosolvan,Mucoangin,盐酸氨溴索,Ambroxol HCl |
Sodium Channel; Autophagy | Autophagy; Membrane transporter/Ion channel |
Ambroxol hydrochloride (Mucosolvan) 是前药溴己辛的活性代谢产物,是一种葡糖脑苷脂酶伴侣,可增加葡糖脑苷脂酶的活性。它可刺激粘液纤毛作用并清除呼吸道中的空气通道,具有强效祛痰作用。它可诱导肺细胞自噬,有用于帕金森氏病和神经性戈谢病的研究潜力。 | |||
T40544 |
NCGC00092410
ML008 |
Glucosidase | Metabolism |
NCGC00092410 (ML008) 是一种葡萄糖脑苷脂酶 (glucocerebrosidase, GC) 抑制剂,IC50 值为 31 nM。可用于由糖脑苷酶基因突变引起的戈谢病的研究。 | |||
T8989 |
NCGC 607
NCGC607 |
Others | Others |
NCGC 607 是一种葡萄糖脑苷酶的非抑制性分子伴侣。 | |||
T60048 |
WAY-640509
|
Glucokinase | Metabolism |
WAY-640509是一种葡糖脑苷酶抑制剂。 | |||
T4280 |
Gcase activator 1
LTI-291,LTI 291,LTI291 |
Glucosidase | Metabolism |
Gcase activator 1 (LTI-291) 是葡糖脑苷脂酶 (Gcase) 的激活剂。 | |||
T8990 | ML266 | Glucokinase | Metabolism |
ML266 是葡萄糖脑苷脂酶 (GCase) 分子伴侣, IC50值为 2.5 µM。ML266与 GCase 结合并将其转运至溶酶体,恢复 GCase 酶的活性。ML266 不会抑制 GCase 的活性。ML266 具有研究戈谢病的潜力。 | |||
T39518 |
PFB-FDGlu
|
Others | Others |
PFB-FDGlu 是一种溶酶体葡糖脑苷脂酶(GCase)底物,具有细胞渗透特异性,裂解后产生荧光素。PFB-FDGlu 通常与流式细胞仪结合使用,以单个细胞为基础测量活细胞中 GCase 的活性。 | |||
T37440 |
C2 Adamantanyl Galactosylceramide (d18:1/2:0)
|
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C2 Adamantanyl Galactosylceramide (d18:1/2:0) (AdaGalCer) is a bioactive sphingolipid. It reduces globotriaosylceramide 3 synthesis from exogenous lactosylceramide in microsomes. AdaGalCer stimulates recombinant glucocerebrosidase activity in a pH-dependent manner. It activates glucocerebrosidase to decrease glucosylceramide accumulation in fibroblasts and lymphoblasts isolated from patients with Gaucher and Fabry disease, respectively. | |||
T80994 |
Therapeutic agent-1
|
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Therapeutic agent-1 是一种杂芳基化合物,用于戈谢病的葡萄糖脑苷脂酶活性酶替代疗法。 | |||
T37928 |
13C6 Glucosylsphingosine (d18:1)
|
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13C6 Glucosylsphingosine (d18:1) is an isotopically enriched form of 1-β-D-glucosylsphingosine (d18:1) that is intended for use as an internal standard for the quantification of 1-β-D-glucosylsphingosine by GC- or LC-MS. 1-β-D-Glucosylsphingosine is a lysolipid derivative of glucosylcerebroside that decreases activity of glucocerebrosidase in LA-N-2 cells in a dose-dependent manner. | |||
T38213 |
1-β-D-Glucosylsphingadienine (d18:2 (4E,8E))
1-β-D-Glucosylsphingadienine (d18:2 (4E,8E)) |
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1-β-D-Glucosylsphingadienine is a glucosylsphingosine, which are deacetylated lysolipid derivatives of glucosylcerebrosides . They are formed when sphingosines undergo glucosidation by UDP-glucose. Glucosylsphingosines completely reduce neurite outgrowth and induce death of LA-N-2 cells at concentrations of 10 and 50 μM, respectively. They also decrease the activity of glucocerebrosidase in LA-N-2 cells in a dose-dependent manner. Glucosylsphingosine levels are elevated in patients with Gaucher'... |
Cat. No. | Product Name | Target | Signaling Pathways |
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T78493 |
Glucosylsphingosine
Lyso-Gb1,Glucopsychosine |
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Glucosylsphingosine(lyso-Gb1)作为糖基神经酰胺的脱酰基产物,同样能被糖脑苷酶所降解。该化合物是监测戈谢病的一个极具潜力、可靠且特异的生物标志物。 |