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Original Article
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Volume 328:1753-1757 June 17, 1993 Number 24
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Autoimmune Protein S Deficiency in a Boy with Severe Thromboembolic Disease
Armando D'Angelo, Patrizia Della Valle, Luciano Crippa, Elisabetta Pattarini, Luigi Grimaldi, and Silvana Vigano D'Angelo

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Protein S is a vitamin K-dependent plasma protein involved in the regulation of the protein C anticoagulant pathway,1 the system responsible for the inactivation of factors Va and VIIIa2. Protein S functions as a cofactor for activated protein C by increasing its affinity for the cell surface3 and by blocking the capacity of factor Xa to protect factor Va from inactivation by activated protein C4; it also enhances the profibrinolytic effects of activated protein C5,6,7.

The congenital deficiency of protein S is associated with an increased risk of recurrent juvenile venous and arterial thromboembolism8,9,10. The association . . . [Full Text of this Article]

Methods

Protein S Determinations

Purification Procedures

Immunoblotting

Detection of Antihuman Protein S IgG by Polyacrylamide Isoelectric Focusing

Enzyme-Linked Immunosorbent Assay (ELISA) for the Detection of Antihuman Protein S IgG in Plasma

Other Procedures

Case Report

Results

Discussion


Source Information

From the Servizio di Coagulazione, Istituto Scientifico H.S. Raffaele (A.D., P.D.V., L.C., E.P., S.V.D.) and Unita di Neuroimmunologia -- Dipartimento Biotecnologie, Clinica Neurologica, Universita degli Studi di Milano (L.M.E.G.), Milan, Italy.

Address reprint requests to Dr. A. D'Angelo at the Servizio di Coagulazione, Istituto Scientifico H.S. Raffaele, Via Olgettina 60, 20132 Milan, Italy.

References


Related Letters:

Autoimmune Protein S Deficiency
Malnick S.D.H., Sthoeger Z.M., D'Angelo A., Crippa L., D'Angelo S. V.
Extract | Full Text  
N Engl J Med 1993; 329:1898, Dec 16, 1993. Correspondence

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