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Serum concentrations of C4 isotypes and factor B in type I C2 deficiency suggest haplotype-dependent quantitative expression of MHC class III complement genes

Truedsson, Lennart LU ; Gullstrand, Birgitta LU ; Jonsson, T and Klint, Cecilia LU (1995) In Experimental and Clinical Immunogenetics 12(2). p.66-73
Abstract
The complement protein C4 exists as two isotypes, C4A and C4B, encoded by genes in the major histocompatibility complex (MHC) class III region. The serum concentrations of C4A4 were lower than those of C4B2 in serum from 19 individuals homozygous for type I C2 deficiency (p < 0.0002). These individuals all had the S042 complotype and most of them were homozygous for the haplotype HLA-B18,S042,DR2. In 14 individuals heterozygous for the C2Q0 gene and with the C4A4, C4B2 phenotype and in 51 individuals with the C4A3, C4B1 phenotype, the isotype concentrations were equal. Factor B concentrations in the C2-deficient individuals were lower than those in individuals with the C4A3, C4B1 phenotype (p < 0.0001). The findings strongly suggest... (More)
The complement protein C4 exists as two isotypes, C4A and C4B, encoded by genes in the major histocompatibility complex (MHC) class III region. The serum concentrations of C4A4 were lower than those of C4B2 in serum from 19 individuals homozygous for type I C2 deficiency (p < 0.0002). These individuals all had the S042 complotype and most of them were homozygous for the haplotype HLA-B18,S042,DR2. In 14 individuals heterozygous for the C2Q0 gene and with the C4A4, C4B2 phenotype and in 51 individuals with the C4A3, C4B1 phenotype, the isotype concentrations were equal. Factor B concentrations in the C2-deficient individuals were lower than those in individuals with the C4A3, C4B1 phenotype (p < 0.0001). The findings strongly suggest that the quantitative expression of C4 isotypes and factor B is MHC haplotype dependent. C4 null alleles cannot be accurately determined by measuring relative C4 isotype serum concentrations. (Less)
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author
organization
publishing date
type
Contribution to journal
publication status
published
subject
in
Experimental and Clinical Immunogenetics
volume
12
issue
2
pages
66 - 73
publisher
Karger
external identifiers
  • pmid:7576717
  • scopus:0029055825
ISSN
0254-9670
language
English
LU publication?
yes
id
4114cc6a-cffe-4703-abdd-10c133eebfcc (old id 1109329)
date added to LUP
2008-07-28 09:59:17
date last changed
2017-07-30 04:39:40
@article{4114cc6a-cffe-4703-abdd-10c133eebfcc,
  abstract     = {The complement protein C4 exists as two isotypes, C4A and C4B, encoded by genes in the major histocompatibility complex (MHC) class III region. The serum concentrations of C4A4 were lower than those of C4B2 in serum from 19 individuals homozygous for type I C2 deficiency (p &lt; 0.0002). These individuals all had the S042 complotype and most of them were homozygous for the haplotype HLA-B18,S042,DR2. In 14 individuals heterozygous for the C2Q0 gene and with the C4A4, C4B2 phenotype and in 51 individuals with the C4A3, C4B1 phenotype, the isotype concentrations were equal. Factor B concentrations in the C2-deficient individuals were lower than those in individuals with the C4A3, C4B1 phenotype (p &lt; 0.0001). The findings strongly suggest that the quantitative expression of C4 isotypes and factor B is MHC haplotype dependent. C4 null alleles cannot be accurately determined by measuring relative C4 isotype serum concentrations.},
  author       = {Truedsson, Lennart and Gullstrand, Birgitta and Jonsson, T and Klint, Cecilia},
  issn         = {0254-9670},
  language     = {eng},
  number       = {2},
  pages        = {66--73},
  publisher    = {Karger},
  series       = {Experimental and Clinical Immunogenetics},
  title        = {Serum concentrations of C4 isotypes and factor B in type I C2 deficiency suggest haplotype-dependent quantitative expression of MHC class III complement genes},
  volume       = {12},
  year         = {1995},
}