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Structural and functional characterization of complement C4 and C1s-like molecules in teleost fish: insights into the evolution of classical and alternative pathways.

There is growing evidence that certain components of complement systems in lower vertebrates are promiscuous in their modes of activation through the classical or alternative pathways. To better understand the evolution of the classical pathway, we have evaluated the degree of functional diversification of key components of the classical and alternative pathways in rainbow trout, an evolutionarily relevant teleost species. Trout C4 was purified in two distinct forms (C4-1 and C4-2), both exhibiting the presence of a thioester bond at the cDNA and protein levels. C4-1 and C4-2 bound in a similar manner to trout IgM-sensitized sheep erythrocytes in the presence of Ca(2+)/Mg(2+), and both C4 molecules equally restored the classical pathway-mediated hemolytic activity of serum depleted of C3 and C4. Reconstitution of activity was dependent on the presence of both C3-1 and C4-1/C4-2 and on the presence of IgM bound to the sheep erythrocytes. A C1s-like molecule was shown to cleave specifically purified C4-1 and C4-2 into C4b, while failing to cleave trout C3 molecules. The C1s preparation was unable to cleave trout factor B/C2 when added in the presence of C3b or C4b molecules. Our results show a striking conservation of the mode of activation of the classical pathway. We also show that functional interchange between components of the classical and alternative pathway in teleosts is more restricted than was anticipated. These data suggest that functional diversification between the two pathways must have occurred shortly after the gene duplication that gave rise to the earliest classical pathway molecules.

Amino Acid Sequence↗

Partial amino acid sequences of the murine fourth component of complement (C4): demonstration of homology with human C4 and identification of the amino-terminal subunit in pro-C4.

Radiolabeled murine C4 and C4 precursor (pro-C4) from cultured peritoneal macrophages were purified by immunoprecipitation and preparative gel electrophoresis. The partial NH2-terminal amino acid sequences of the isolated subunits demonstrated that murine C4 alpha, beta-, and gamma-chains all show sequence homology with the corresponding subunits of human C4; of the 10 residues identified in murine C4, all are identical to those in human C4. These data extend to the primary structural level the homology between the murine serum substance (Ss) protein and human C4. Comparisons of the amino acid sequences of murine pro-C4 and the constituent polypeptide chains of secreted C4 indicate that pro-C4 and the C4 beta-chain have an identical amino acid sequence. Both molecules have lysine at position 1, leucine at positions 4, 5, and 6, and phenylalanine at position 7. No sequence homology was found between pro-C4 and either the alpha- or gamma-subunits. These results define the beta-chain as the NH2-terminal subunit in the C4 precursor molecules.

Amino Acid Sequence↗

Human major histocompatibility complex contains a new cluster of genes between the HLA-D and complement C4 loci.

A new cluster of genes has been defined in the human Major Histocompatibility Complex class III region. The seven novel genes, G12 to G18, are localised in a 160 kb segment of DNA extending from the complement gene cluster towards HLA-DR. The genes were identified by isolation of cDNA clones using cosmid genomic inserts as hybridisation probes, and by the detection of the corresponding transcripts in Northern blot analysis. Characterisation of the cosmid genomic DNA inserts, in conjunction with pulsed field gel electrophoresis analysis of uncloned DNA, for the presence of clustered sites for infrequently cutting restriction endonucleases has revealed that at least 5 of the 7 genes are associated with HTF-islands. These unmethylated CpG-rich sequences are frequently found at the 5' ends of ubiquitously expressed genes. Together with previously published data 36 genes have now been defined in a 680kb stretch of DNA within the MHC. With one gene approximately every 20kb of DNA this represents the most densely packed region of the human genome so far characterised, and is of major significance in relation to the mapping and sequence analysis of the rest of the genome.

Blotting, Northern↗

Prolonged clinically asymptomatic evolution after HIV-1 infection is marked by the absence of complement C4 null alleles at the MHC.

The length of time after which persons infected with HIV-1 progress to AIDS is variable. Certain alleles at the MHC have been shown to influence negatively the clinical outcome of HIV-1-infected persons and to be associated with special clinical manifestations. We investigated the MHC class I, class II and class III antigens in 54 Caucasian HIV-1-infected persons. The MHC profile of individuals with a prolonged period before AIDS is marked by a lower frequency of C4 null alleles.

Acquired Immunodeficiency Syndrome↗

Family studies of complement C4 and HLA in man.

At least 12 different C4 gene products with a three band pattern have been identified after electrophoresis of sera pretreated with neuraminidase. Segregation analysis showed at least 12 different C4 haplotypes (or supergenes), of which five represent a single gene product and seven are duplications each composed of an F and an S gene. The data analyzed with respect to linkage showed one recombinant between the C4 and HLAB loci in 154 meioses giving a map distance of C4 HLAB of 0.6 cM. Another recombinant between the C4 and the HLAD loci was found in 101 meioses giving a map distance of C4 HLAD of 1.0 cM. Linkage disequilibrium was found between at least eight C4 haplotypes and certain alleles at the HLAB as well as the HLAD loci. Examinations of 15 families selected through a proband with HLAA 25, HLAB 18 and C2Q0 showed that in almost all cases a slight variant of the C4 supergene F3S2 followed the haplotype HLAA25 HLAB18 C2Q0. No associations were found between the two duplications of C4F3 C4S2 and C4F3 C4S1 and the loci. These findings may indicate that these C4 haplotypes were the original ones preceding the other C4 haplotypes.

Adult↗

Determination of circulating immune complexes, C3 and C4 complement components and anti-DNA antibody in different classes of lupus nephritis.

Circulating immune complexes (CIC) were measured in 237 sera from children who underwent a renal biopsy during the course of systemic lupus erythematosus. CIC-positive sera contained a lower mean level of C3 but not C4. Anti-DNA antibody was similar in CIC-positive and negative sera. The World Health Organization classification of lupus nephritis was used to categorize the biopsies. CIC, C3, C4 and anti-DNA antibody were determined to assess whether they correlated with the severity of renal lesions. Of 25 sera obtained at renal biopsy from patients with classes 2, 3 and 4, 16 were positive for CIC. C3 was significantly lower in classes 3 and 4 than in class 2. C4 was reduced and anti-DNA antibody was present in classes 2, 3 and 4. Determination of the level of C3 but not C4, CIC or anti-DNA antibody correlates with the severity of lupus nephritis seen on renal biopsies. Nevertheless, performance of a renal biopsy is preferred.

Antibodies, Anti-Idiotypic↗

Genetic studies of complement C4 in man.

A C4 variant found in about 5% of the population is described. The fast-moving part of this variant is governed by an allele (Fx) codominant to F. The Fx allele is in very strong linkage disequilibrium with HLA-B17 as the linkage disequilibrium parameter accounted for nearly 100% of the haplotype frequency of B17,Fx. The strong association is also evidenced by the study of 11 families segregating for the Fx allele. There was no instance of recombination between C4 and HLA in 36 informative meioses.

Adult↗

The endogenous retroviral insertion in the human complement C4 gene modulates the expression of homologous genes by antisense inhibition.

Intron 9 contains the complete endogenous retrovirus HERV-K(C4) as a 6.4-kb insertion in 60% of human C4 genes. The retroviral insertion is in reverse orientation to the C4 coding sequence. Therefore, expression of C4 could lead to the transcription of an antisense RNA, which might protect against exogenous retroviral infections. To test this hypothesis, open reading frames from the HERV sequence were subcloned in sense orientiation into a vector allowing expression of a beta-galactosidase fusion protein. Mouse L cells which had been stably transfected with either the human C4A or C4B gene both carrying the HERV insertion (LC4 cells), and L(Tk-) cells without the C4 gene were transiently transfected either with a retroviral construct or with the wild-type vector. Expression was monitored using an enzymatic assay. We demonstrated that (1) HERV-K(C4) antisense mRNA transcripts are present in cells constitutively expressing C4, (2) expression of retroviral-like constructs is significantly downregulated in cells expressing C4, and (3) this downregulation is further modulated in a dose-dependent fashion following interferon-gamma stimulation of C4 expression. These results support the hypothesis of a genomic antisense strategy mediated by the HERV-K(C4) insertion as a possible defense mechanism against exogenous retroviral infections.

Animals↗

Deficiency of the fourth component of complement (C4): a family case.

In this report, an apparently healthy 38-year-old woman with a remarkably low serum C4 value is described together with other family members who had moderately low serum C4. Plasma C4 typing disclosed that the proband inherited two C4B "null" haplotypes. In addition, Southern blot analysis of the C4 gene indicated that the C4A gene was partially deleted on one of these two haplotypes in the proband. We thus concluded that a de novo deletion on the inherited half-null haplotype was the likeliest cause of the low C4 level.

Adult↗

Genetic polymorphism of complement C4 in the dog.

Plasma samples from 129 dogs have been typed using an immunofixation technique to define the polymorphism of canine C4. The mode of inheritance was inferred by study of four large pedigrees. In contrast to the situation in man, canine C4 may be encoded by a single locus. At least five different co-dominant structural alleles have been detected. A deficiency allele has not been found as yet. Because there are associations between C4 allotypes and spontaneous autoimmune disease in man, the well defined polymorphism of canine C4 may provide an excellent model for understanding of the pathogenetic mechanisms of autoimmune disease.

Alleles↗

[Hereditary deficiency of the 4th component of complement (C4) associated with a lupic syndrome].

A 2 year-old girl presented with bacterial meningitis followed by a lupus erythematosus syndrome consisting of erythematous rash, Raynaud's phenomenon and mutilating cicatricia atrophy; high speckled antinuclear antibodies and anti-Sm and anti-Ro antibodies titers; a selective absence of C4; moderate mesangial proliferation with IgM and C1q mesangial deposits. Study of the family revealed a linkage of C4 deficiency with the HLA A1 B17/BfF haplotype.

Antibodies, Antinuclear↗