title: Functional assay of protein S in 70 patients with congenital and acquired disorders.
abstract: A functional assay for the selective measurement of the active form of protein S in plasma, based on the prolongation of an APTT, was previously developed. This assay is sensitive, reproducible and specific, not affected by other clotting factors including FVIII. This method was applied to the measurement of protein S activity in congenital and acquired disorders. Results of protein S activity were compared to those of total and free antigen measured by ELISA. In 30 controls, there was an excellent correlation between protein S activity and free antigen. In patients with inflammatory disease, protein S activity and free antigen were normal, despite high levels of both C4b-binding protein and total protein S antigen. In dicoumarol-treated patients, protein S activity was lower than free antigen due to the presence of acarboxylated forms. Surprisingly, in liver cirrhosis, free antigen was only slightly decreased whereas protein S activity was significantly reduced. In 23 patients with congenital deficiency, protein S activity was consistently decreased, from less than 5% to 60% and showed good correlation with the free antigen. This functional assay allows the rapid diagnosis of congenital or acquired deficiency of protein S.
authors.affiliation: Laboratoire d'Hématologie, Hôpital Bicêtre, Le Kremlin-Bicêtre, France.
authors.forename: M
authors.initials: M
authors.lastname: Wolf
authors.forename: C
authors.initials: C
authors.lastname: Boyer-Neumann
authors.forename: C
authors.initials: C
authors.lastname: Leroy-Matheron
authors.forename: J L
authors.initials: JL
authors.lastname: Martinoli
authors.forename: G
authors.initials: G
authors.lastname: Contant
authors.forename: J
authors.initials: J
authors.lastname: Amiral
authors.forename: D
authors.initials: D
authors.lastname: Meyer
authors.forename: M J
authors.initials: MJ
authors.lastname: Larrieu
mesh_terms: D000328:Adult; D002352:Carrier Proteins / Q000302:isolation & purification; D003169:Complement Inactivator Proteins; D004797:Enzyme-Linked Immunosorbent Assay; D015943:Factor Va / Q000378:metabolism; D005260:Female; D030342:Genetic Diseases, Inborn / Q000097:blood; D006023:Glycoproteins / Q000097:blood / Q000172:deficiency* / Q000302:isolation & purification; D006801:Humans; D007249:Inflammation / Q000097:blood; D008107:Liver Diseases / Q000097:blood; D008297:Male; D008875:Middle Aged; D010314:Partial Thromboplastin Time; D010375:Pedigree; D011486:Protein C / Q000378:metabolism; D017293:Protein S
chemical_list: D002352:Carrier Proteins; D003169:Complement Inactivator Proteins; D006023:Glycoproteins; D011486:Protein C; D017293:Protein S; D015943:Factor Va