A case report of deficiency in an inhibitor of calcium-dependent association of protein S with C4B-binding protein suggested by a modified crossed immunoelectrophoresis

Kaoru Hatanaka, Xiang An Li, Ling Guo, Toshiyuki Sakata, Judith A. Gillissen, Akira Yoshioka, Akira Yamamoto

Research output: Contribution to journalArticlepeer-review

2 Scopus citations

Abstract

We have experienced a coagulation factor VIII-deficient patient whose plasma has normal protein S (PS) activity and masses of free PS and its bound form in complex with C4b-binding protein (C4BP). Although the patient's plasma showed a normal ratio of free PS to PS-C4BP complex in the presence of 5 mM EDTA, the plasma gave an abnormally retarding major C4BP peak together with a major PS peak in the crossed immunoelectrophoresis (CIE) in the presence of 2 mM CaCl2. It was revealed that the major peak was formed by a mixture of PS-C4BP complex and free form. The addition of normal human plasma (NHP) to the patient's plasma inhibited the retardation of the major PS-C4BP complex. These suggest that the patient's plasma lacks some component(s) to inhibit Ca2+-dependent association of PS with C4BP.

Original languageEnglish
Pages (from-to)643-654
Number of pages12
JournalThrombosis Research
Volume74
Issue number6
DOIs
StatePublished - Jun 15 1994

Bibliographical note

Funding Information:
PS is a vitamin K-dependent plasma protein (1) which serves as a cofactor for active form of protein C (PC) to inactivate coagulation factors Va and VIIIa (2-4). In human plasma, PS exists in two forms; one is free form which is active, the other is bound form which is in equimolar complex with C4BP (5). C4BP binds C4b and promotes its inhibition by factor I of complement Key words: protein S, C4b-binding protein, calcium, factor VIII, serum amyloid P component, P,-glycoprotein I. Corresponding author: Dr. Kaoru Hatanalca,D epartment of Etiology/Pathophysiology, National Cardiovascular Center Research Institute, 5-7-l Fujishiro-dai, Suita City, Osaka 565, Japan. tvisiting scholar from Taishan Medical College, Taian, Shandong, P. R. of China, fmancially supported by the fellowships of the Japan Cardiovascular Research Foundation and Japan Health Sciences Foundation. SVisiting scholar from Taian Central Hospital, Taian, Shandong, P. R. of China.

Keywords

  • C4b-binding protein
  • calcium
  • factor VIII
  • protein S
  • serum amyloid P component
  • β-glycoprotein I

ASJC Scopus subject areas

  • Hematology

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