Natural人C1 Inactivator protein (ab90388)

概述

描述

  • 性质Natural
  • 来源Native
  • 氨基酸序列
    • 种属Human

技术指标

Our Abpromise guarantee covers the use of ab90388 in the following tested applications.

The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.

  • 应用

    SDS-PAGE

  • 纯度> 95 % SDS-PAGE.

  • 形式Liquid
  • Concentration information loading...

制备和贮存

  • 稳定性和存储

    Shipped on dry ice. Upon delivery aliquot and store at -80ºC. Avoid freeze / thaw cycles.

    Preservative: None
    Constituents: 250mM Sodium chloride, 20mM Potassium phosphate, pH 7.0

常规信息

  • 别名
    • C1 esterase inhibitor
    • C1 Inh
    • C1 inhibiting factor
    • C1 inhibitor
    • C1-inhibiting factor
    • C1IN
    • C1Inh
    • C1NH
    • complement component 1 inhibitor
    • esterase inhibitor
    • HAE1
    • HAE2
    • IC1_HUMAN
    • Plasma protease C1 inhibitor
    • Serine (or cysteine) proteinase inhibitor clade G member 1
    • serine/cysteine proteinase inhibitor clade G member 1
    • Serpin G1
    • serpin peptidase inhibitor, clade G (C1 inhibitor), member 1
    • SERPING1
    see all
  • 功能Activation of the C1 complex is under control of the C1-inhibitor. It forms a proteolytically inactive stoichiometric complex with the C1r or C1s proteases. May play a potentially crucial role in regulating important physiological pathways including complement activation, blood coagulation, fibrinolysis and the generation of kinins. Very efficient inhibitor of FXIIa. Inhibits chymotrypsin and kallikrein.
  • 疾病相关Defects in SERPING1 are the cause of hereditary angioedema (HAE) [MIM:106100]; also called hereditary angioneurotic edema (HANE). HAE is an autosomal dominant disorder characterized by episodic local subcutaneous edema and submucosal edema involving the upper respiratory and gastrointestinal tracts. HAE due to C1 esterase inhibitor deficiency is comprised of two clinically indistinguishable forms. In HAE type 1, representing 85% of patients, serum levels of C1 esterase inhibitor are less than 35% of normal. In HAE type 2, the levels are normal or elevated, but the protein is non-functional.
  • 序列相似性Belongs to the serpin family.
  • 翻译后修饰Highly glycosylated (49%) with N- and O-glycosylation.
    Can be proteolytically cleaved by E.coli stcE.
  • 细胞定位Secreted.
  • Information by UniProt

Natural Human C1 Inactivator protein (ab90388)参考文献

ab90388 has not yet been referenced specifically in any publications.

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