製品の概要

製品の詳細

  • 由来
    Native
  • 由来
    Native
  • アミノ酸配列
    • 生物種
      Cow
    • 配列の追加情報
      Source = fresh citrated bovine plasma

特性

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.

  • アプリケーション

    SDS-PAGE

    Functional Studies

  • 製品の状態
    Liquid
  • Concentration information loading...

前処理および保存

  • 保存方法および安定性

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

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

関連情報

  • 別名
    • 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
  • 機能
    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.
  • 組織特異性
    Synthesized primarily in the liver and secreted in plasma.
  • 関連疾患
    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.
  • 配列類似性
    Belongs to the peptidase S1 family.
    Contains 2 EGF-like domains.
    Contains 1 Gla (gamma-carboxy-glutamate) domain.
    Contains 1 peptidase S1 domain.
  • ドメイン
    Calcium binds to the gamma-carboxyglutamic acid (Gla) residues and, with stronger affinity, to another site, beyond the Gla domain.
  • 翻訳後修飾
    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.
  • 細胞内局在
    Secreted.
  • Information by UniProt

参考文献

ab62547 has not yet been referenced specifically in any publications.

レビューと Q&A

There are currently no Customer reviews or Questions for ab62547.
Please use the links above to contact us or submit feedback about this product.

Please note: All products are "FOR RESEARCH USE ONLY AND ARE NOT INTENDED FOR DIAGNOSTIC OR THERAPEUTIC USE"

登録