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Science 2 April 1976:
Vol. 192. no. 4234, pp. 56 - 59
DOI: 10.1126/science.1083071
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Articles

Science, Vol 192, Issue 4234, 56-59
Copyright © 1976 by American Association for the Advancement of Science

articles

Carbohydrate deficiency of the factor VIII/von Willebrand factor Protein in von Willebrand's disease variants

HR Gralnick, BS Coller, and Y Sultan

Study of the normal human factor VIII/von Willebrand factor reveals a macromolecular glycoprotein composed of apparently identical subunits. This purified glycoprotein has procoagulant, antigen, and von Willebrand factor activities. In three patients with a variant of the von Willebrand's disease syndrome, their factor VIII/von Willebrand factor protein was present in normal amounts and had normal procoagulant and antigen activities; however, this protein was deficient in both carbohydrate and von Willebrand factor activity. The carbohydrate portion of the factor VIII/von Willebrand factor glycoprotein is of major importance in its interactions with platelets or the blood vessel wall, or both.


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Evolution and Classification of Cystine Knot-Containing Hormones and Related Extracellular Signaling Molecules.
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Plasma Cofactors of Platelet Function: Correlation with Diabetic Retinopathy and Hemoglobins Ala-c Studies in Diabetic Patients and Normal Persons.
B. S. COLLER, R. N. FRANK, R. C. MILTON, and H. R. GRALNICK (1978)
Ann Intern Med 88, 311-316
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Antihemophilic Factor (Factor VIII).
O. D. RATNOFF (1978)
Ann Intern Med 88, 403-409
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Sickle Cell Trait and Hematuria Associated with von Willebrand Syndromes.
J. I. BRODY, S. P. LEVISON, and C.-J. JUNG (1977)
Ann Intern Med 86, 529-533
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Factor VIII.
H. R. GRALNICK, B. S. COLLER, N. R. SHULMAN, J. C. ANDERSEN, and M. HILGARTNER (1977)
Ann Intern Med 86, 598-616
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