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Genetic deficiency of the fourth component of complement (C4) in Wistar rats. 1977

C M Arroyave, and R M Levy, and J S Johnson

A chance observation has led to the discovery of single component complement deficiency in Wistar rats. During serial total haemolytic activity (CH50) determinations on 10 male rats, 2 were consistently found to have values 20% or less than that of the others. An additional 30 males (200--220 g) and 20 females (180--200 g) were screened for CH50 and the defect was found in 10 of the males but none of the females. Mixtures of sera of high and low haemolytic activity gave intermediate values between the two populations arguing against the presence of an inhibitor of the complement system. Single component analysis revealed the defect to reside in the fourth component of complement (C4). Highly purified human C4 completely reconstituted the haemolytic activity of the complement deficient rat serum. A linear relationship between C4 and CH50 titres was observed. Data from preliminary selective breeding experiments point toward an autosomal recessive mode of inheritance. Animals with intermediate values were observed indicating a gene dosage effect with which the heterozygote may be recognized.

UI MeSH Term Description Entries
D008297 Male Males
D001947 Breeding The production of offspring by selective mating or HYBRIDIZATION, GENETIC in animals or plants. Breedings
D003169 Complement Inactivator Proteins Serum proteins that negatively regulate the cascade process of COMPLEMENT ACTIVATION. Uncontrolled complement activation and resulting cell lysis is potentially dangerous for the host. The complement system is tightly regulated by inactivators that accelerate the decay of intermediates and certain cell surface receptors. Complement Cytolysis Inhibiting Proteins,Complement Cytolysis Inhibitor Proteins,Complement Inactivating Proteins,Serum Complement Inactivators,Complement Inactivators, Serum,Inactivating Proteins, Complement,Inactivator Proteins, Complement,Inactivators, Serum Complement,Proteins, Complement Inactivating,Proteins, Complement Inactivator
D003181 Complement C4 A glycoprotein that is important in the activation of CLASSICAL COMPLEMENT PATHWAY. C4 is cleaved by the activated COMPLEMENT C1S into COMPLEMENT C4A and COMPLEMENT C4B. C4 Complement,C4 Complement Component,Complement 4,Complement C4, Precursor,Complement Component 4,Pro-C4,Pro-complement 4,C4, Complement,Complement Component, C4,Complement, C4,Component 4, Complement,Component, C4 Complement,Pro C4,Pro complement 4
D005260 Female Females
D005808 Genes, Recessive Genes that influence the PHENOTYPE only in the homozygous state. Conditions, Recessive Genetic,Genetic Conditions, Recessive,Recessive Genetic Conditions,Condition, Recessive Genetic,Gene, Recessive,Genetic Condition, Recessive,Recessive Gene,Recessive Genes,Recessive Genetic Condition
D006461 Hemolysis The destruction of ERYTHROCYTES by many different causal agents such as antibodies, bacteria, chemicals, temperature, and changes in tonicity. Haemolysis,Extravascular Hemolysis,Intravascular Hemolysis,Extravascular Hemolyses,Haemolyses,Hemolyses, Extravascular,Hemolyses, Intravascular,Hemolysis, Extravascular,Hemolysis, Intravascular,Intravascular Hemolyses
D000818 Animals Unicellular or multicellular, heterotrophic organisms, that have sensation and the power of voluntary movement. Under the older five kingdom paradigm, Animalia was one of the kingdoms. Under the modern three domain model, Animalia represents one of the many groups in the domain EUKARYOTA. Animal,Metazoa,Animalia
D000917 Antibody Formation The production of ANTIBODIES by proliferating and differentiated B-LYMPHOCYTES under stimulation by ANTIGENS. Antibody Production,Antibody Response,Antibody Responses,Formation, Antibody,Production, Antibody,Response, Antibody,Responses, Antibody
D051381 Rats The common name for the genus Rattus. Rattus,Rats, Laboratory,Rats, Norway,Rattus norvegicus,Laboratory Rat,Laboratory Rats,Norway Rat,Norway Rats,Rat,Rat, Laboratory,Rat, Norway,norvegicus, Rattus

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