Origin of the fourth component of complement related Chido and Rodgers blood group antigens.
We have reviewed the relationship between C4 and its related blood group and discussed the mechanisms whereby a fragment of C4 could become attached to erythrocytes (E). We hypothesize that there is chronic fluid-phase activation of C4 by either C1 to form C4b or spontaneous cleavage of the thioester to form iC4. These activated molecules bind to E. Proteolytic degradation of the bound C4b or iC4 would leave a covalently attached fragment of C4 on E and thereby give rise to the Ch and Rg blood group antigens. This system is of further immunopathologic interest since this 'normal' activation or turnover of C4 is closely regulated. In patients deficient in regulatory proteins, this spontaneous or normal turnover of C4 and C3 may initiate a pathologic condition.
Atkinson
J P
JP
Howard Hughes Medical Institute Laboratories, Washington University School of Medicine, St. Louis, Mo.
Chan
A C
AC
Karp
D R
DR
Killion
C C
CC
Brown
R
R
Spinella
D
D
Shreffler
D C
DC
Levine
R P
RP
D000818:Animals; D001789:Blood Group Antigens / Q000276:immunology; D003167:Complement Activation; D003181:Complement C4 / Q000235:genetics / Q000276:immunology / Q000378:metabolism; D004912:Erythrocytes / Q000276:immunology; D006801:Humans; D010446:Peptide Fragments / Q000378:metabolism; D011951:Receptors, Complement / Q000378:metabolism; D017463:Receptors, Complement 3b
D001789:Blood Group Antigens; D003181:Complement C4; D010446:Peptide Fragments; D011951:Receptors, Complement; D017463:Receptors, Complement 3b