Heterogeneity of Sugar Composition of Factor VIII/ von Willebrand Factor in von Willebrand's Disease: Analysis by Crossed Affinoimmunoelectrophoresis Using Lectin (Ricinus communis Agglutinin-120)

Summary

The nature of sugar chain of factor VTII/von Willebrand factor in plasma of normal subjects and patients with von Willebrand’s disease (vWd) was examined by crossed affinoimmunoelectrophoresis using anti-human factor VIII rabbit serum, with inserted Ricinus communis agglutinin-120 (RCA-120) agarose layer (RCA – CIE). Molecular weights of factor VlU-related antigen (VIIIR: Ag) were estimated by SDS polyacrylamide gel electrophoresis — crossed affinoimmunoelectrophoresis (SDS PAGE – RCA – CIE).

VIIIR :Ag, in normal plasma and in classical form of vWd, showed two precipitin peaks on RCA – CIE. The slower moving component of VIIIR :Ag with molecular weights over 3×10⁶ daltons from normal subjects and patients with classical form of vWd showed a high affinity for RCA-120. The faster moving component of VIIIR: Ag below 3×10⁶ daltons from the abovementioned subjects and patients with a variant form (Type IIA) showed a very weak affinity for RCA-120.

These results suggested that all of VIIIR: Ag in these variant cases may have a deficiency of galactose residues reactive with RCA, in addition to an incomplete polymerization of VIIIR: Ag, similar to that of the faster moving component of normal subjects.

Keywords

von Willebrand’s disease - Factor Vlll/von Willebrand factor - Sugar composition - Crossed affinoimmunoelectrophoresis - Ricinus communis agglutinin-120

• References

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