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Factor VIII Arg2304 --> His binds to phosphatidylserine and is a functional cofactor in the factor X-ase complex.

Publication ,  Journal Article
Lewis, DA; Moore, KD; Ortel, TL
Published in: Thromb Haemost
February 2001

Four factor VIII light chain constructs containing hemophilia A mutations at R2304 and R2307 were prepared and expressed in mammalian cells. These mutations are located in a putative phosphatidylserine binding site identified by peptide studies (spanning amino acids 2303-2332). The levels of all four mutants in conditioned medium were significantly less than wild type by immunoprecipitation and ELISA. R2304H and wild type factor VIII light chains were concentrated by cation exchange chromatography from medium. R2304H and wild type factor VIII light chains bound immobilized phosphatidylserine similarly. The reconstituted cofactor activity of R2304H factor VIII light chain was slightly greater than wild type factor VIII light chain. These results are consistent with the recently reported crystal structure of factor VIII C2 domain that suggests R2304H is not directly involved in phospholipid binding. The observed clinical phenotype is probably due to decreased circulating levels of a functional protein.

Duke Scholars

Published In

Thromb Haemost

ISSN

0340-6245

Publication Date

February 2001

Volume

85

Issue

2

Start / End Page

260 / 264

Location

Germany

Related Subject Headings

  • Transfection
  • Protein Binding
  • Phosphatidylserines
  • Neoplasm Proteins
  • Mutagenesis, Site-Directed
  • Humans
  • Hemophilia A
  • Factor VIII
  • Cysteine Endopeptidases
  • Cell Line
 

Citation

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Lewis, D. A., Moore, K. D., & Ortel, T. L. (2001). Factor VIII Arg2304 --> His binds to phosphatidylserine and is a functional cofactor in the factor X-ase complex. Thromb Haemost, 85(2), 260–264.
Lewis, D. A., K. D. Moore, and T. L. Ortel. “Factor VIII Arg2304 --> His binds to phosphatidylserine and is a functional cofactor in the factor X-ase complex.Thromb Haemost 85, no. 2 (February 2001): 260–64.
Lewis, D. A., et al. “Factor VIII Arg2304 --> His binds to phosphatidylserine and is a functional cofactor in the factor X-ase complex.Thromb Haemost, vol. 85, no. 2, Feb. 2001, pp. 260–64.
Journal cover image

Published In

Thromb Haemost

ISSN

0340-6245

Publication Date

February 2001

Volume

85

Issue

2

Start / End Page

260 / 264

Location

Germany

Related Subject Headings

  • Transfection
  • Protein Binding
  • Phosphatidylserines
  • Neoplasm Proteins
  • Mutagenesis, Site-Directed
  • Humans
  • Hemophilia A
  • Factor VIII
  • Cysteine Endopeptidases
  • Cell Line