目录号 | 产品详情 | 靶点 | |
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T9189 | Complement System | ||
Iptacopan hydrochloride (LNP023 hydrochloride) 是一种可口服、高效和高选择性的因子 B 抑制剂,IC50 为 10 nM。 LNP023 显示与人因子 B 的直接、可逆和高亲和力结合,KD 为 7.9 nM。 | |||
T8150 | PARP Endogenous Metabolite | ||
Nudifloramide (1-Methyl-6-oxo-1,6-dihydropyridine-3-carboxamide) 是烟酰胺-腺嘌呤二核苷酸(NAD) 降解的一种最终产物,可显著抑制PARP-1活性。 | |||
T19866 | OAT MRP | ||
Indican (Indoxyl-β-D-glucoside) 是吲哚酚的一种糖苷,染料靛蓝和靛玉红的前体。它的主要代谢物是硫酸吲哚酚 (IS)。其中 IS 是尿毒症毒素,是有机阴离子转运蛋白1 (OAT 1)、OAT 3 和多药耐药相关蛋白4 (MRP 4) 的底物/抑制剂。 | |||
T19498 | Endogenous Metabolite | ||
p-Cresyl sulfate potassium (p-Tolyl sulfate potassium salt) 是一种尿毒症毒素,能够与原型蛋白质结合。 | |||
T21977 | Others | ||
p-Cresyl Sulfate 是主要的尿毒症毒素,存在于慢性肾病患者的血液,来源于肝脏中酪氨酸和苯丙氨酸的代谢物。 | |||
T7523 | Others Endogenous Metabolite | ||
Guanidinosuccinic acid 是一种含氮代谢物,在尿毒症患者的血清和尿液中水平升高。 | |||
T5289 | Others Endogenous Metabolite | ||
N-Acetyl-L-arginine dihydrate (N-Ac-L-arg-OH) 是内源性代谢产物的一种。 | |||
T76867 | Complement System | ||
Ravulizumab (ALXN1210) 是一种靶向补体因子 5 的人源化单克隆抗体,它以高亲和力特异性结合人补体蛋白 C5,可阻断补体激活。Ravulizumab 可用于预防和治疗阵发性睡眠性血红蛋白尿症、非典型溶血性尿毒症综合征和重症肌无力。 | |||
T8509 | Complement System | ||
Danicopan (ACH-4471) 是一种可口服的小分子 D 因子选择性抑制剂,对人 D 因子具有高结合亲和力,Kd 值为 0.54 nM。 它可抑制补体替代途径 (APC) 的活性,具有阻断阵发性夜间血红蛋白尿 (PNH) 和非典型溶血性尿毒症综合征 (aHUS) 的补体替代途径的潜力。 | |||
T8053 | Endogenous Metabolite | ||
N-Acetyl-L-arginine (Ac-Arg-OH) 是一种胍基诺化合物,被发现存在于血液渗析性肾不足儿童患者血清中的。 |
目录号 | 产品名/同用名 | 种属 | 表达系统 | ||
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TMPY-01075 | Von Willebrand Factor/vWF Protein, Human, Recombinant (His) | Human | CHO | ||
Von Willebrand Factor (VWF) is a multimeric glycoprotein involved in hemostasis in blood, binds receptors on the surface of platelets and in connective tissue, thereby mediating the adhesion of platelets to sites of vascular injury. From studies it appears that VWF protein uncoils under these circumstances, decelerating passing platelets. VWF protein is deficient or defective in von Willebrand disease (VWD) and is involved in a large number of other diseases, including thrombosis, thrombotic thrombocytopenic purpura, Stroke, Heyde's syndrome, possibly hemolytic-uremic syndrome and so on.
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TMPJ-01306 | CFHR5 Protein, Human, Recombinant (His) | Human | Human Cells | ||
Complement factor H-related protein 5(FHR-5 for short), is a secreted protein which contains 9 Sushi (CCP/SCR) domains. It is expressed by the liver and secreted in plasma. The pattern of the deposits is similar to other complement components, suggesting that FHR-5 may play a role in complement activation and regulation. Defects in CFHR5 have been found in patients with atypical hemolytic uremic syndrome and may contribute to the disease. In contrast to typical hemolytic uremic syndrome, atypical forms have a poorer prognosis, with higher death rates and frequent progression to end-stage renal disease.
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TMPK-00790 | Complement factor H/CFH Protein, Human, Recombinant (His) | Human | HEK293 | ||
Factor H is the major soluble inhibitor of complement, where its binding to self markers (i.e., particular glycan structures) prevents complement activation and amplification on host surfaces. Not surprisingly, mutations and polymorphisms that affect recognition of self by factor H are associated with diseases of complement dysregulation, such as age-related macular degeneration and atypical haemolytic uremic syndrome. In addition, pathogens (i.e., non-self) and cancer cells (i.e., altered-self) can hijack factor H to evade the immune response.
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TMPY-02023 | CD46 Protein, Human, Recombinant (His) | Human | HEK293 | ||
CD46, also known as Membrane Cofactor Protein (MCP), is a complement regulatory protein. CD46 is a type 1 membrane protein that plays an important inhibitory role in the complement system. CD46 is expressed in white blood cells, platelets, epithelial cells, and fibroblasts. Human CD46 shares 5% amino acid sequence identity with mouse and rat CD46. The importance of CD46 to complement regulation is underscored by the observation that genetic loss of CD46 leads to development of atypical hemolytic-uremic syndrome (aHUS), a disease characterized by uncontrolled complement activation. CD46 is implicated in the development and/or progression of selected cancer types.
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TMPY-05829 | Thrombomodulin Protein, Human, Recombinant (His) | Human | HEK293 | ||
Thrombomodulin, also known as THBD (CD141), is an integral membrane protein that reduces blood coagulation by converting thrombin to an anticoagulant enzyme from a procoagulant enzyme. Thrombomodulin is expressed on the surface of endothelial cells and serves as a cofactor for thrombin. It is also expressed on human mesothelial cell, monocyte and a dendritic cell subset. Thrombomodulin functions as a cofactor in the thrombin-induced activation of protein C in the anticoagulant pathway by forming a 1:1 stoichiometric complex with thrombin. Thrombomodulin also regulates C3b inactivation by factor I. Mutations in the thrombomodulin gene have also been reported to be associated with atypical hemolytic-uremic syndrome.
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TMPY-03271 | Thrombomodulin Protein, Rat, Recombinant (His) | Rat | HEK293 | ||
Thrombomodulin, also known as THBD (CD141), is an integral membrane protein that reduces blood coagulation by converting thrombin to an anticoagulant enzyme from a procoagulant enzyme. Thrombomodulin is expressed on the surface of endothelial cells and serves as a cofactor for thrombin. It is also expressed on human mesothelial cell, monocyte and a dendritic cell subset. Thrombomodulin functions as a cofactor in the thrombin-induced activation of protein C in the anticoagulant pathway by forming a 1:1 stoichiometric complex with thrombin. Thrombomodulin also regulates C3b inactivation by factor I. Mutations in the thrombomodulin gene have also been reported to be associated with atypical hemolytic-uremic syndrome.
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TMPY-04129 | Complement factor H/CFH Protein, Mouse, Recombinant (His) | Mouse | HEK293 | ||
Complement factor H, also known as H factor 1, and CFH, is a sialic acid containing glycoprotein that plays an integral role in the regulation of the complement-mediated immune system that is involved in microbial defense, immune complex processing, and programmed cell death. Factor H protects host cells from injury resulting from unrestrained complement activation. CFH regulates complement activation on self cells by possessing both cofactor activity for the Factor I mediated C3b cleavage, and decay accelerating activity against the alternative pathway C3 convertase, C3bBb. CFH protects self cells from complement activation but not bacteria/viruses. Due to the central role that CFH plays in the regulation of complement, there are many clinical implications arrising from aberrant CFH activity. Mutations in the Factor H gene are associated with severe and diverse diseases including the rare renal disorders hemolytic uremic syndrome (HUS) and membranoproliferative glomerulonephritis (MPGN) also termed dense deposit disease (DDD), membranoproliferative glomuleronephritis type II or dense deposit disease, as well as the more frequent retinal disease age related macular degeneration (AMD). In addition to its complement regulatory activities, factor H has multiple physiological activities and 1) acts as an extracellular matrix component, 2) binds to cellular receptors of the integrin type, and 3) interacts with a wide selection of ligands, such as the C-reactive protein, thrombospondin, bone sialoprotein, osteopontin, and heparin.
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TMPY-04141 | Complement factor H/CFH Protein, Mouse, Recombinant (hFc) | Mouse | HEK293 | ||
Complement factor H, also known as H factor 1, and CFH, is a sialic acid containing glycoprotein that plays an integral role in the regulation of the complement-mediated immune system that is involved in microbial defense, immune complex processing, and programmed cell death. Factor H protects host cells from injury resulting from unrestrained complement activation. CFH regulates complement activation on self cells by possessing both cofactor activity for the Factor I mediated C3b cleavage, and decay accelerating activity against the alternative pathway C3 convertase, C3bBb. CFH protects self cells from complement activation but not bacteria/viruses. Due to the central role that CFH plays in the regulation of complement, there are many clinical implications arrising from aberrant CFH activity. Mutations in the Factor H gene are associated with severe and diverse diseases including the rare renal disorders hemolytic uremic syndrome (HUS) and membranoproliferative glomerulonephritis (MPGN) also termed dense deposit disease (DDD), membranoproliferative glomuleronephritis type II or dense deposit disease, as well as the more frequent retinal disease age related macular degeneration (AMD). In addition to its complement regulatory activities, factor H has multiple physiological activities and 1) acts as an extracellular matrix component, 2) binds to cellular receptors of the integrin type, and 3) interacts with a wide selection of ligands, such as the C-reactive protein, thrombospondin, bone sialoprotein, osteopontin, and heparin.
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