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PubMed1986

Detection of both isotypes of complement C4, C4A and C4B, in normal human glomeruli.

Monoclonal antibodies reactive against the complement C4A and C4B isotypic components were used in an immunoperoxidase technique for the histological study of normal human renal tissue. Prominent staining with both antibodies was seen in the mesangial areas of all normal kidney sections investigated. Occasional staining of arteriolar walls of the same tissues, however, was also observed. In contrast, no mesangial staining was seen using monoclonal antibodies reactive against other 'early' complement components, such as C1q and C3. Specificity of the glomerular staining with the anti-C4 reagents was demonstrated in two patients possessing only the C4A serum component but lacking genetically the C4B locus products. As would be predicted, glomerular staining with the anti-C4A reagent, but not anti-C4B, was clearly demonstrable. It is concluded that both isotypes of complement C4 are present in normal human glomeruli and thus might be operative for normal mesangial function.

Antibodies, Monoclonal↗
PubMed1984

Rare variant of complement C4 is seen in high frequency in patients with primary glomerulonephritis.

59 unselected patients with primary glomerulonephritis were phenotyped for alleles of the MHC-linked complement genes, C4A, C4B, and BF. A rare variant of the C4B locus, C4B*2.9, was found in 25% of these patients compared with only 2% of the normal population--a relative risk of 22.1 for glomerulonephritis in individuals with this variant. Subdivision of patients by histological classification of glomerulonephritis revealed a significant association of C4B*2.9 with the membranoproliferative form. There were no significant associations between primary glomerulonephritis or its subtypes and the other HLA markers tested.

Alleles↗
PubMed2002

The follicular dendritic cell restricted epitope, FDC-M2, is complement C4; localization of immune complexes in mouse tissues.

We have identified the murine follicular dendritic cell (FDC) marker, FDC-M2, recognized by monoclonal antibody mAb209, as complement component C4. Consistent with this, FDC-M2 was detectable at sites of immune complex-mediated inflammatory disease. Analysis of FDC-M2 distribution in complement-deficient mice highlighted the differences in immune complex clearance between these mice, andshowed that a population of uncharacterized FDC-M2+ reticular and perivascular cells in the spleen, distinct from FDC, are also involved in immune complex capture and possibly retention. These results demonstrate that mAb209, in addition to its role as an FDC marker, is a valuable reagent for the analysis of complement deposition in vivo.

Animals↗
PubMed1990

A case of first trimester prenatal diagnosis of 21-hydroxylase deficiency with human complement C4 cDNA probe.

Early prenatal diagnosis of 21-hydroxylase (21-OHase) deficiency would enable treatment to be done to protect the fetus from masculinization and/or life-threatening adrenal crisis at birth. We report here the prenatal diagnosis of 21-OHase deficiency with human complement component C4 cDNA to probe DNA from chorionic villi at 10 weeks of gestation. Southern analysis with human C4 cDNA identified TaqI restriction fragment length polymorphisms (RFLPs) in the family. Family analysis with these RELPs showed that the fetus was not affected at greater than 99% probability, because the frequency of recombination between the 21-OHase B gene and the C4 gene would be extremely low.

Adrenal Hyperplasia, Congenital↗
PubMed1989

Determination of deletion sizes in the MHC-linked complement C4 and steroid 21-hydroxylase genes by pulsed-field gel electrophoresis.

In man, the genes encoding the complement component C4 (C4A, C4B) of the immune system and the steroid 21-hydroxylase enzyme (CYP21A, CYP21B) of adrenal steroid biosynthesis are located in the major histocompatibility complex (MHC). Frequent gene deletions and duplications have been described in the C4 and CYP21 genes, particularly in patients with autoimmune diseases and congenital adrenal hyperplasia. Here we report the determination of deletion sizes in 11 chromosomes with six different deletions. The deletions spanned the C4A+CYP21A, C4B+CYP21A, and C4B+CYP21B gene pairs as determined by standard Southern blot analysis. The deletion size fell within the range of 30-38 kb in all the chromosomes, as determined by pulsed-field gel electrophoresis. Because the deletion sizes in most other gene clusters are more heterogeneous, the results suggest the involvement of a specific mechanism in the generation of C4+CYP21 deletions.

Chromosome Deletion↗
PubMed2003

Dancing with complement C4 and the RP-C4-CYP21-TNX (RCCX) modules of the major histocompatibility complex.

The number of the complement component C4 genes varies from 2 to 8 in a diploid genome among different human individuals. Three quarters of the C4 genes in Caucasian populations have the endogenous retrovirus, HERV-K(C4), in the ninth intron. The remainder does not. The C4 serum proteins are highly polymorphic and their concentrations vary from 100 to approximately 1000 microg/ml. There are two distinct classes of C4 protein, C4A and C4B, which have diversified to fulfill (a) the opsonization/immunoclearance purposes and (b) the well-known complement function in the killing of microbes by lysis and neutralization, respectively. Many infectious and autoimmune diseases are associated with complete or partial deficiency of C4A and/or C4B. The adverse effects of high C4 gene dosages, however, are just emerging, as the concepts of human C4 genetics are revised and accurate techniques are applied to distinguish partial deficiencies from differential expression caused by unequal C4A and C4B gene dosages and gene sizes. This review attempts to dissect the sophisticated genetics of complement C4A and C4B. The emphases are on the qualitative and quantitative diversities of C4 genotypes and phenotypes. The many allotypic variants and the processed products of human and mouse C4 proteins are described. The modular variation of C4 genes together with the serine/threonine nuclear kinase gene RP, the steroid 21-hydroxylase CYP21, and extracellular matrix protein TNX (RCCX modules) are investigated for the effects on homogenization of C4 protein polymorphisms, and on the unequal genetic crossovers that knocked out the functions of CYP21 and/or TNX. Furthermore, the influence of the endogenous retrovirus HERV-K(C4) on C4 gene expression and the dispersal of HERV-K(C4) family members in the human genome are discussed.

Animals↗
PubMed1985

Fourth component of complement (C4) polymorphism in human orthotopic liver transplantation.

C4 polymorphism was investigated in 13 orthotopic liver transplantations. It could be shown that recipient C4 phenotype disappears after transplantation and is replaced by donor phenotype on days 10-19 posttransplantation. This indicates that C4 is mainly produced in the liver. The delayed appearance of donor C4 phenotype compared with other complement components produced in the liver cannot be explained by different rates of synthesis or serum protein levels. The limited number of patients investigated in this study does not permit assessment of the role of C4, C3, Bf, and HLA-A, B, and DR in orthotopic liver transplantation.

Alleles↗
PubMed1986

Difference in the biological properties of the two forms of the fourth component of human complement (C4).

The two forms of human C4 were compared in their haemolytic activity and in their capacity to mediate inhibition of immune complex precipitation in human C4 deficient sera. Whereas haemolysis by C4B was 3.2 fold more efficient than by C4A, C4A was 1.7 fold more efficient at inhibiting immune precipitation than C4B. Thus the biological properties of the two forms of human C4 are different and it is suggested that C4A is mainly involved in immune complex clearance reactions.

Antigen-Antibody Complex↗
PubMedDate not supplied

Partial deficiency of the fourth component of human complement (C4) and autoantibody directed against C4 in a patient with SLE.

The finding of dramatically depressed levels of C4 in a 17-year old patient with severe systemic lupus erythematosus (SLE) prompted a genetic study of her family. This study revealed the existence of a partial C4 deficiency; we found the presence of a C4A3,C4BQo haplotype which was transmitted by the mother to each of her children. This patient possessed, in her serum, an autoantibody with anti-C4 specificity. The immunochemical characterization of this autoantibody revealed that it was IgM and belonged to the immunoconglutinin family. We have studied the effects of this autoantibody on the formation and dissociation kinetics of classical C3-convertase.

Adolescent↗