ELK Biotechnology FA7 (light chain, Cleaved-Ala61) rabbit pAb

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FA7 (light chain, Cleaved-Ala61) rabbit pAb

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Background

• Catalytic activity: Selective cleavage of Arg-Ile bond in factor X to form factor Xa.
• Disease association: Defects in F7 cause factor VII deficiency (MIM:227500), a rare hereditary hemorrhagic disease. Severity ranges from intracerebral hemorrhage to mild mucosal bleeding.
• Function: Initiates the extrinsic pathway of blood coagulation. Factor VII circulates as a zymogen and is activated to VIIa by minor proteolysis. In the presence of tissue factor and calcium ions, VIIa converts factor X to Xa and factor IX to IXa.
• Pharmaceutical relevance: Available as Niastase or Novoseven (Novo Nordisk), used for treating bleeding episodes in hemophilia A or B patients with inhibitors to factors VIII or IX.
• Polymorphism: Individuals with the Q allele (Gln-413) may have decreased susceptibility to myocardial infarction.
• Post-translational modifications (PTM):
  • Iron and 2-oxoglutarate dependent 3-hydroxylation of aspartate and asparagine is (R) stereospecific within EGF domains.
  • Vitamin K-dependent carboxylation of glutamate residues enables calcium binding.
• Similarity:
  • Belongs to peptidase S1 family.
  • Contains 1 Gla (gamma-carboxy-glutamate) domain.
  • Contains 1 peptidase S1 domain.
  • Contains 2 EGF-like domains.
• Subunit structure: Heterodimer of light and heavy chains linked by a disulfide bond.
• Tissue specificity: Plasma.

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