APPARENT DOMINANT AND RECESSIVE INHERITANCE OF VON-WILLEBRANDS DISEASE WITHIN THE SAME KINDREDS - POSSIBLE BIOCHEMICAL-MECHANISMS

In 2 kindreds with von Willebrand''s disease patients the pattern of inheritance exhibited recessive and dominant features. This phenomenon may be associated with the fact that the factor VIII related antigen/Willebrand factor exists in normal plasma as a series of homologous oligomers. Those with the highest MW exhibit the most potent platelet-related biological activities. The variable inheritance patterns in some von Willebrand''s disease kindreds may be due to interaction between maternally and paternally derived protomers or related biosynthetic pathways that produce various series of oligomers in the offspring.