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.
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 Ca(2+)-dependent association of PS with C4BP.
Hatanaka
K
K
Department of Etiology/Pathophysiology, National Cardiovascular Center, Osaka, Japan.
Li
X A
XA
Guo
L
L
Sakata
T
T
Gillissen
J A
JA
Yoshioka
A
A
Yamamoto
A
A
D000328:Adult; D002118:Calcium / Q000502:physiology*; D002352:Carrier Proteins / Q000737:chemistry*; D015935:Complement C4b; D003169:Complement Inactivator Proteins; D004587:Electrophoresis, Agar Gel; D006023:Glycoproteins; D006467:Hemophilia A / Q000097:blood*; D006801:Humans; D007123:Immunoelectrophoresis, Two-Dimensional; D008297:Male; D017293:Protein S / Q000737:chemistry*; D011951:Receptors, Complement / Q000737:chemistry*
D002352:Carrier Proteins; D003169:Complement Inactivator Proteins; D006023:Glycoproteins; D017293:Protein S; D011951:Receptors, Complement; D015935:Complement C4b; D002118:Calcium