Evidence for a serum inhibitor of Clq.
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Acidic mucopolysaccharides enhanced C1INA activity against C1s. The presence of heparin in the reaction mixture of C1INA and C1s markedly enhanced the inactivation of C1s by potentiating C1INA activity. Treatment of serum with C1s resulted in the inactivation of C4 hemolytic activity, but this inactivation of C4 by C1s was prevented by the presence of heparin presumably due to the enhancement of C1INA activity normally present in serum. The other acidic mucopolysaccharides, chondroitin sulfates A, B and C, were also effective on potentiating C1INA activity against C1s, but they were less active than heparin. The enhancement of C1INA activity by heparin was not observed in the reaction between C1INA and plasmin.
The presence of circulating immune complexes in freshly drawn sera of patients with various forms of malignancies was detected by the 125I-Clq deviation test of Sobel et al. More than 50% of the 459 cancer sera showed a high inhibition of 125I-Clq uptake by sensitized sheep erythrocytes when compared with sera of 50 healthy laboratory personnel. The levels were compared with levels of total hemolytic complement and immunochemical determinations of Cl1 and C3. A correlation between high levels of circulating immune complexes and low levels of Clq was suggested. These immune complexes were separated by sucrose density gradient ultracentrifugation at low pH and were found to be heavier than 19S. Fluctuation of levels of immune complexes was evident when serial samples from the same patient were tested. Decrease of levels of immune complexes and a concomitant increase of Clq were detected after Calmette-Gueérin bacillus and autologous tumor cell treatment in some melanoma patients.
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Extremely low C4 values were found in a 65-year-old man with relapsing arthritis and skin lesions of many years duration of the scalp, face, hands and feet together with painful ulcerations of the toes and fingers. The discovery was made during an exacerbation, but the deficiency of C4 persisted in repeated controls after remission. The clinical findings in connection with these low C4 values are in congruence with the diagnosis of inherited deficiency of C4.
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