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

doi:10.1016/0049-3848(94)90220-8

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

Physiology

Research output: Contribution to journal › Article › peer-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 language	English
Pages (from-to)	643-654
Number of pages	12
Journal	Thrombosis Research
Volume	74
Issue number	6
DOIs	https://doi.org/10.1016/0049-3848(94)90220-8
State	Published - 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.

Funding

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.

Funders	Funder number
Japan Cardiovascular Research Foundation
Taishan Medical College
Japan Health Sciences Foundation

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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10.1016/0049-3848(94)90220-8

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@article{cceeef8a8fc548319f0fce7e0849e64e,

title = "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",

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.",

keywords = "C4b-binding protein, calcium, factor VIII, protein S, serum amyloid P component, β-glycoprotein I",

author = "Kaoru Hatanaka and Li, {Xiang An} and Ling Guo and Toshiyuki Sakata and Gillissen, {Judith A.} and Akira Yoshioka and Akira Yamamoto",

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.",

year = "1994",

month = jun,

day = "15",

doi = "10.1016/0049-3848(94)90220-8",

language = "English",

volume = "74",

pages = "643--654",

number = "6",

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T1 - 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

AU - Hatanaka, Kaoru

AU - Li, Xiang An

AU - Guo, Ling

AU - Sakata, Toshiyuki

AU - Gillissen, Judith A.

AU - Yoshioka, Akira

AU - Yamamoto, Akira

N1 - 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.

PY - 1994/6/15

Y1 - 1994/6/15

N2 - 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.

AB - 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.

KW - C4b-binding protein

KW - calcium

KW - factor VIII

KW - protein S

KW - serum amyloid P component

KW - β-glycoprotein I

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U2 - 10.1016/0049-3848(94)90220-8

DO - 10.1016/0049-3848(94)90220-8

M3 - Article

C2 - 8091406

AN - SCOPUS:0028236710

SN - 0049-3848

VL - 74

SP - 643

EP - 654

JO - Thrombosis Research

JF - Thrombosis Research

IS - 6

ER -

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

Abstract

Bibliographical note

Funding

Keywords

ASJC Scopus subject areas

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