Inhibitory sequences within the B-domain stabilize circulating factor V in an inactive state

J Biol Chem. 2007 May 18;282(20):15033-9. doi: 10.1074/jbc.M701315200. Epub 2007 Mar 26.

Abstract

Blood coagulation factor V circulates as a procofactor with little or no procoagulant activity. It is activated to factor Va by thrombin following proteolytic removal of a large central B-domain. Although this reaction is well studied, the mechanism by which bond cleavage and B-domain release facilitate the transition to the active cofactor state has not been defined. Here we show that deletion or substitution of specific B-domain sequences drives the expression of procoagulant function without the need for proteolytic processing. Conversion to the constitutively active cofactor state is related, at least in part, to a cluster of amino acids that is highly basic and well conserved across the vertebrate lineage. Our findings demonstrate that discrete sequences in the B-domain serve to stabilize the inactive procofactor state, with proteolysis primarily functioning to remove these inhibitory constraints. These unexpected results provide new insight into the mechanism of factor V activation.

Publication types

  • Research Support, N.I.H., Extramural
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Amino Acid Sequence / genetics
  • Amino Acid Substitution
  • Coenzymes / genetics
  • Coenzymes / metabolism
  • Enzyme Activation / genetics
  • Factor V / genetics
  • Factor V / metabolism*
  • Humans
  • Mutation, Missense
  • Protein Processing, Post-Translational / physiology*
  • Protein Structure, Tertiary / genetics
  • Sequence Deletion

Substances

  • Coenzymes
  • Factor V