Overview

Description

  • Nature
    Native
  • Source
    Native
  • Amino Acid Sequence
    • Species
      Rat

Associated products

Specifications

Our Abpromise guarantee covers the use of ab95131 in the following tested applications.

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

  • Biological activity
    17.7 U/mg (One unit is equivalent to the Factor IX activity in one milliliter of normal human plasma.) Activity is measured using a Factor IX clotting assay.
  • Applications

    SDS-PAGE

    Functional Studies

  • Purity
    > 95 % SDS-PAGE.
    ab95131 is prepared from fresh frozen plasma by a combination of conventional procedures and immunoaffinity chromatography.
  • Form
    Liquid
  • Concentration information loading...

Preparation and Storage

  • Stability and Storage

    Shipped at 4°C. Store at -20°C. Stable for 12 months at -20°C.

    Preservative: None
    Constituents: 50% Glycerol

    This product is an active protein and may elicit a biological response in vivo, handle with caution.

General Info

  • Alternative names
    • 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
  • Function
    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.
  • Tissue specificity
    Synthesized primarily in the liver and secreted in plasma.
  • Involvement in disease
    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.
  • Sequence similarities
    Belongs to the peptidase S1 family.
    Contains 2 EGF-like domains.
    Contains 1 Gla (gamma-carboxy-glutamate) domain.
    Contains 1 peptidase S1 domain.
  • Domain
    Calcium binds to the gamma-carboxyglutamic acid (Gla) residues and, with stronger affinity, to another site, beyond the Gla domain.
  • Post-translational
    modifications
    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.
  • Cellular localization
    Secreted.
  • Information by UniProt

References

ab95131 has not yet been referenced specifically in any publications.

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

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