Recombinant Human Factor VIII protein (ab158403)

Overview

Description

  • Nature
    Recombinant
  • Source
    Wheat germ
  • Amino Acid Sequence
    • Species
      Human
    • Sequence
      MRIQDPGKVFFGNVDSSGIKHNIFNPPIIARYIRLHPTHYSIRSTLRMEL MGCDLNSCSMPLGMESKAISDAQITASSYFTNMFATWSPSKARLHLQGRS NAWRPQVNNPKEWLQVDFQKTMKVTGVTTQGVKSLLTSMYVKEFLISSSQ DGHQWTLFFQNGKVKVFQGNQDSFTPVVNSLDPPLLTRYLRIHPQSWVHQ IALRMEVLGCEAQDLY
    • Amino acids
      1 to 216
    • Tags
      GST tag N-Terminus

Specifications

Our Abpromise guarantee covers the use of ab158403 in the following tested applications.

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

  • Applications

    Western blot

    ELISA

  • Form
    Liquid
  • Additional notes
    Protein concentration is above or equal to 0.05 mg/ml.
  • Concentration information loading...

Preparation and Storage

  • Stability and Storage

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

    pH: 8.00
    Constituents: 0.31% Glutathione, 0.79% Tris HCl

General Info

  • Alternative names
    • AHF
    • Antihemophilic factor
    • Coagulation factor VIII
    • coagulation factor VIII, procoagulant component
    • coagulation factor VIIIc
    • DXS1253E
    • F8
    • F8b
    • F8c
    • FA8_HUMAN
    • factor VIII F8B
    • Factor VIIIa light chain
    • FactorVIII
    • FVIII
    • Hema
    • Hemophilia A
    • Hemophilia, classic
    • OTTHUMP00000061446
    • OTTHUMP00000196174
    • Procoagulant component
    see all
  • Function
    Factor VIII, along with calcium and phospholipid, acts as a cofactor for factor IXa when it converts factor X to the activated form, factor Xa.
  • Involvement in disease
    Defects in F8 are the cause of hemophilia A (HEMA) [MIM:306700]. A disorder of blood coagulation characterized by a permanent tendency to hemorrhage. About 50% of patients have severe hemophilia resulting in frequent spontaneous bleeding into joints, muscles and internal organs. Less severe forms are characterized by bleeding after trauma or surgery. Note=Of particular interest for the understanding of the function of F8 is the category of CRM (cross-reacting material) positive patients (approximately 5%) that have considerable amount of F8 in their plasma (at least 30% of normal), but the protein is non-functional; i.e., the F8 activity is much less than the plasma protein level. CRM-reduced is another category of patients in which the F8C antigen and activity are reduced to approximately the same level. Most mutations are CRM negative, and probably affect the folding and stability of the protein.
  • Sequence similarities
    Belongs to the multicopper oxidase family.
    Contains 3 F5/8 type A domains.
    Contains 2 F5/8 type C domains.
    Contains 6 plastocyanin-like domains.
  • Domain
    Domain F5/8 type C 2 is responsible for phospholipid-binding and essential for factor VIII activity.
  • Post-translational
    modifications
    Sulfation on Tyr-1699 is essential for binding vWF.
  • Cellular localization
    Secreted > extracellular space.
  • Information by UniProt

Images

  • ab158403 on a 12.5% SDS-PAGE stained with Coomassie Blue.

References

ab158403 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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