Natural牛Factor IX protein (ab62547)

概述

描述

  • 性质Natural
  • 来源Native
  • 氨基酸序列
    • 种属Cow
    • 额外的序列信息Source = fresh citrated bovine plasma

技术指标

Our Abpromise guarantee covers the use of ab62547 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

    Functional Studies

  • 形式Liquid
  • Concentration information loading...

制备和贮存

  • 稳定性和存储

    Shipped at 4°C. Upon delivery aliquot and store at -20°C. Avoid freeze / thaw cycles.

    Preservative: None
    Constituents: 50% Glycerol, 50% H2O

常规信息

  • 别名
    • Christmas Disease
    • Christmas factor
    • Coagulant factor IX
    • Coagulation factor 9
    • Coagulation factor IX
    • Coagulation factor IXa heavy chain
    • F9
    • FA9_HUMAN
    • Factor 9
    • Factor IX Deficiency
    • FactorIX
    • FIX
    • Haemophilia B
    • HEMB
    • MGC129641
    • MGC129642
    • P19
    • Plasma Thromboplastic Component
    • Plasma thromboplastin component
    • PTC
    see all
  • 功能Factor IX is a vitamin K-dependent plasma protein that participates in the intrinsic pathway of blood coagulation by converting factor X to its active form in the presence of Ca(2+) ions, phospholipids, and factor VIIIa.
  • 组织特异性Synthesized primarily in the liver and secreted in plasma.
  • 疾病相关Defects in F9 are the cause of recessive X-linked hemophilia B (HEMB) [MIM:306900]; also known as Christmas disease.
    Note=Mutations in position 43 (Oxford-3, San Dimas) and 46 (Cambridge) prevents cleavage of the propeptide, mutation in position 93 (Alabama) probably fails to bind to cell membranes, mutation in position 191 (Chapel-Hill) or in position 226 (Nagoya OR Hilo) prevent cleavage of the activation peptide.
    Defects in F9 are the cause of thrombophilia due to factor IX defect (THR-FIX) [MIM:300807]. A hemostatic disorder characterized by a tendency to thrombosis.
  • 序列相似性Belongs to the peptidase S1 family.
    Contains 2 EGF-like domains.
    Contains 1 Gla (gamma-carboxy-glutamate) domain.
    Contains 1 peptidase S1 domain.
  • 结构域Calcium binds to the gamma-carboxyglutamic acid (Gla) residues and, with stronger affinity, to another site, beyond the Gla domain.
  • 翻译后修饰Activated by factor XIa, which excises the activation peptide.
    The iron and 2-oxoglutarate dependent 3-hydroxylation of aspartate and asparagine is (R) stereospecific within EGF domains.
  • 细胞定位Secreted.
  • Information by UniProt

Natural Cow Factor IX protein (ab62547)参考文献

ab62547 has not yet been referenced specifically in any publications.

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