PubMed1975
Members of six unrelated families from Japan, France, Belgium and Poland were studied in parallel. Major immunological features characteristic of the phenotype produced by the Cis AB complex are the following: 1) The red cell A reactivity is close to normal, is beyond the values of agglutination scores by Helix and by anti-A from B; likewise, with percent agglutination measurements, A reactive appears hiher than that of A2B cells; one sample only is slightly detected by anti-A from Dolichos. 2) The B reactivity, on the contrary, is lower than that of normal AB cells. A single sample is detected by anti-B from A1. All samples are well detected by anti-B from AW, Aend, Ax, Am but none is detected by anti-B from ABx, Cis AB, or by an auto-anti-B. Under standard conditions, percent aggutination is around 80, very close to that of normal AB cells, thus differentiating Cis AB from AB3 (some of which only reach this figure), and from ABx which are very far from this value. 3) An abnormally high reactivity to anti-H antibody is observed, higher than that of normal A2B, similar to that of A2 red cells. 4) Among secretors, A substance is found to be normal or in excess, H substance is in excess, while B substance is only detected by Cis AB red cells inhibition. 5)An anti-B antibody was identified in the samples studied; however, we recently received from Germany a Cis AB samples, the serum of which did not contain anti-B antibody. By these main characteristics, the studied samples seem to be identical; however, agglutination kinetics and thermodynamic methods show that they differ by their reaction with a same anti-B antibody in standard conditions. The reactive structures of the various samples are indeed different from one family to another. The main point is that identical values were observed in all samples within a same family. Thus, the various Cis AB can be considered as different families mutants.