Phenotypes Associated with This Genotype

Genotype
MGI:3581652

• Allelic Composition: Cd55^tm1Song/Cd55^tm1Song
• Genetic Background: involves: 129S6/SvEvTac * C57BL/6

• ♀: phenotype observed in females
• ♂: phenotype observed in males
• N: normal phenotype

immune system

immune system phenotype ( J:52968 )

N • in vivo, mutant erythrocytes display no increase in spontaneous (autologous) C3 deposition relative to wild-type erythrocytes

abnormal complement pathway ( J:52968 )

• notably, mutant erythrocytes show increased susceptibility to heterologous complement hemolysis when tested with human or guinea pig sera

• increased susceptibility to heterologous complement lysis also correlates with increased complement activation at the C3 level

• when antibody-sensitized mouse erythrocytes are incubated with factor B-depleted human serum, mutant erythrocytes show a significant increase in hemolysis, indicating inhibition of human classical pathway complement activation

• in vitro, mutant erythrocytes show increased autologous C3 deposition from bystander and antibody-initiated complement activation relative to wild-type erythrocytes

• no homologous hemolysis is observed despite a significantly high level of C3 fixation on mutant erythrocytes

impaired complement alternative pathway ( J:52968 )

• in vitro, mutant erythrocytes show impaired regulation of bystander complement fixation initiated by the alternative pathway complement activator zymosan, resulting in increased autologous C3 deposition

impaired complement classical pathway ( J:52968 )

• in vitro, mutant erythrocytes show impaired regulation of antibody-triggered classical pathway complement activation, resulting in increased autologous C3 deposition

hematopoietic system

hematopoietic system phenotype ( J:52968 )

N • homozygotes are viable, fertile and display neither overt abnormalities nor signs of hemolytic anemia

N • in addition, total erythrocyte and leukocyte counts remain normal relative to wild-type counts