C1s Proenzyme

货号: C414536
有货
级别和纯度: EnzymoPure™ ? EnzymoPure™ —— 阿拉丁的高质量酶解决方案系列。当酶纯度和明确活性决定检测或工艺性能时使用。 PBS Only ? 仅 PBS 配方 —— 以磷酸盐缓冲液供应,不含其他添加剂。当需要不含稳定剂/载体的洁净 PBS 缓冲液时使用。 1.0 mg/mL,0.22 µm filtered
生物活性
>80 % C1s binds 1:1 with excess C1-INH after activation.
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规格
库存
价格
数量
10μg
C414536-10μg
期货 Stock Image
¥999.90
50μg
C414536-50μg
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¥2,999.90
250μg
C414536-250μg
期货 Stock Image
¥8,899.90
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为什么选择此级别

EnzymoPure™,PBS Only 级 ,适用于对基线干扰要求严格的色谱和分析工作流程。

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储存与运输

-80℃储存,避免反复冻融。特低温运输 。请查阅批次 COA 获取详细规格。

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质量文档

SDS、COA、产品数据表及规格说明书均可下载。可通过批号查询获取批次 COA。

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文献证明

在色谱分析、有机合成和交叉偶联反应领域已被 0 篇同行评审文献引用。

概述


Protein Purity

>90% by SDS PAGE


Extinction Coeff.

A280 nm = 0.96 at 1.0 mg/mL for pure C1s


Molecular Weight

86,000 Da (1 chain)


General Description

C1s proenzyme is a single chain 86,000 dalton protein that is the native form ofC1s enzyme. C1s is a subunit of the C1 complex which is the first complement component in the cascade referred to as the classical pathway of complement. C1s proenzyme is an inactive zymogen until C1 is activated. C1 complex binds to and is activated by antigen-antibody complexes (immune complexes) yielding C1r enzyme. C1r enzyme in the C1 complex activates C1s proenzyme generating C1s enzyme. C1 complex is a non-covalent calcium-dependent complex of one C1q, two C1r and two C1s molecules. C1q binds through two or more of its six arms to the Fc domains of IgG or IgM. The binding of multiple arms to immune complexes causes the two C1r proteins in the complex (protease zymogens) to activate producing two proteases that cleave and activate the two C1s proenzymes in the complex (Morikis, D. and Lambris, J.D. (2005)). This activation of C1s proenzyme is caused by cleavage into the two chain C1s enzyme with 58,000 and 28,000 dalton fragments. 

Activated C1s enzyme cleaves complement component C4 releasing C4a and initiating covalent attachment of C4b to the activating surface. Activated C1s also cleaves C2 and the larger fragment of C2 binds to the surface-attached C4b forming C4b,C2a, the C3/C5 convertase of the classical pathway.


Physical Characteristics & Structure

C1s proenzyme is a high molecular weight (86,000 daltons), single chain, zymogen of a trypsin-like protease. C1s is present in plasma at 31 ug/mL. C1s proenzyme is activated by C1r enzyme (Dodds, A.W. and Sim, R.B. editors (1997)). Two C1r molecules form a C1r-C1r complex in the presence of calcium. C1r-C1r subequently forms a stable complex with two C1s molecules in the presence of calcium. This tetramer can exist in solution, but in the presence of C1q it binds forming the C1 complex which is stable in the presence of calcium. C1s proenzyme circulates in blood in this 766,000 dalton C1 complex. C1r self-activation, and subsequent C1s activation, is controlled by a weak association of C1r with C1 esterase inhibitor (C1-INH) when it is in the C1 complex and similar stabilization occurs with purified C1r. C1s and C1r enzymes, however, are irreversibly inactivated by binding to C1-INH (Ziccardi, R.J. (1982)).


Function

C1s proenzyme can be used in the presence of calcium to form the C1 complex with C1q and C1r proenzyme. C1s proenzyme has no proteolytic activity and must be converted to C1s enzyme by incubation with C1r enzyme to be active. C1s enzyme can be used to activate C4 or C2 in the fluid phase. In the presence of Mg++ activated C4b and C2a can form fluid phase C4b,C2a which is the C3/C5 convertase of the classical pathway. The C1 complex made from C1s proenzyme will, after activation to C1s enzyme, activate C4 and C2. This occurs after binding of C1 to a cell bearing antibodies such as EA (Dodds, A.W. and Sim, R.B. editors (1997); Morgan, B.P. ed. (2000)). EA are sheep erythrocytes with rabbit IgM anti-sheep erythrocytes antibodies bound to their surface (Morgan, B.P. ed. (2000)).


Assays

The activity of C1s proenzyme is checked by first activating it with C1r enzyme and subsequently measuring its ability to bind to the protease inhibitor C1 inhibitor. It may also be used to form the C1 complex composed of one C1q, two C1r and two C1s molecules and this may be assayed as described for C1 complex.


Applications

See section titled Function above.


Regulation

Activation of C1s proenzyme in the C1 complex is regulated indirectly by C1-INH. C1r self-activation, and subsequent C1s activation, is controlled by a weak association of C1r with C1 esterase inhibitor (C1-INH) when it is in the C1 complex (Ziccardi, R.J. (1982)). Once activated, C1s enzyme is rapidly inactivated by C1-INH. 


Genetics

The EMBL/Genbank cDNA accession number for C1s proenzyme is J04080. The genes for C1r and C1s are closely linked and located on chromosome 12p13.


Deficiencies

Deficiencies of each of the three components of C1 have been found (Ross, G.D. (1986)). C1r and C1s deficient patients are prone to systemic lupus erythematosus (SLE) and recurrent pyogenic infections (Rother, K., et al. (1998)). They lack classical pathway function and may or may not exhibit antigen in blood. 


Diseases

See section titled Deficiencies above.

规格

产品名称
C1s Proenzyme
规格或纯度
EnzymoPure™,PBS Only,1.0 mg/mL,0.22 µm filtered
生物活性
>80 % C1s binds 1:1 with excess C1-INH after activation.
分子类型
蛋白质
产品线
生物产品
储存与运输
储存缓冲液
50 mM sodium phosphate, 130 mM NaCl, pH 7.2
浓度
1.0 mg/mL,0.22 µm filtered
储存条件
-80℃储存,避免反复冻融
运输条件
特低温运输

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