PMID: 6976355Dec 1, 1981Paper

Characterization of the defect in activation of factor IX Chapel Hill by human factor XIa

The Journal of Clinical Investigation
K M BraunsteinH R Roberts

Abstract

Factor IXChapel Hill (Factor IXCH), an abnormal Factor IX molecule isolated from the plasma of a patient with mild hemophilia B, has previously been shown to exhibit delayed activation by Factor XIa and calcium. In this study, we have found that Factor IXCH is cleaved upon incubation with human Factor XIa and calcium; however, cleavage of this protein is not observed by sodium dodecyl sulfate (SDS) polyacrylamide gel electrophoresis under nonreducing conditions. Under reducing conditions, the rate of disappearance of the zymogen parallels both the appearance of the heavy chain and the generation of clotting activity. In addition, a protein band that migrates with an apparent molecular weight of 45,000 also increases in parallel with clotting activity. Factor IXCH and normal Factor IX (Factor IXN), after incubation with Factor XIa and calcium, were subjected to amino terminal sequence analysis. Activated Factor IXN is cleaved at an arginine-alanine (Arg-Ala) bond and an arginine-valine (Arg-Val) bond as demonstrated by formation of the three amino terminal sequences corresponding to the amino terminal of the light chain, heavy chain, and activation peptide. However, activated Factor IXCH has only two amino terminal sequences, co...Continue Reading

References

Dec 1, 1977·Proceedings of the National Academy of Sciences of the United States of America·B Osterud, S I Rapaport
Oct 1, 1979·Proceedings of the National Academy of Sciences of the United States of America·K KatayamaK Titani
Jun 1, 1978·The Journal of Clinical Investigation·R G Di ScipioE W Davie
Apr 1, 1967·Lancet·C Hougie, J J Twomey
Jul 1, 1973·British Journal of Haematology·W R NealH R Roberts
Mar 1, 1967·European Journal of Biochemistry·P Edman, G Begg

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Citations

Jul 1, 1983·Proceedings of the National Academy of Sciences of the United States of America·C M NoyesR L Lundblad
May 1, 1998·The Annals of Thoracic Surgery·C R Smith
May 30, 1998·The Journal of Thoracic and Cardiovascular Surgery·T B SpanierA M Schmidt
Aug 1, 1982·Thrombosis Research·M J GriffithH R Roberts
May 1, 1985·Archives of Biochemistry and Biophysics·D M MonroeM J Griffith
Aug 13, 1996·Biochemistry·A S WolbergD W Stafford

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