Determination of the loss of function complement C4 exon 29 CT insertion using a novel paralog-specific assay in healthy UK and Spanish populations.

Genetic variants resulting in non-expression of complement C4A and C4B genes are common in healthy European populations and have shown association with a number of diseases, most notably the autoimmune disease, systemic lupus erythematosus. The most frequent cause of a C4 "null" allele, fo...

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Main Authors: Lora Boteva, IMAGEN, Yee Ling Wu, Josefina Cortes-Hernández, Javier Martin, Timothy J Vyse, Michelle M A Fernando
Format: Article
Language:English
Published: Public Library of Science (PLoS) 2011-01-01
Series:PLoS ONE
Online Access:https://journals.plos.org/plosone/article/file?id=10.1371/journal.pone.0022128&type=printable
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author Lora Boteva
IMAGEN
Yee Ling Wu
Josefina Cortes-Hernández
Javier Martin
Timothy J Vyse
Michelle M A Fernando
author_facet Lora Boteva
IMAGEN
Yee Ling Wu
Josefina Cortes-Hernández
Javier Martin
Timothy J Vyse
Michelle M A Fernando
author_sort Lora Boteva
collection DOAJ
description Genetic variants resulting in non-expression of complement C4A and C4B genes are common in healthy European populations and have shown association with a number of diseases, most notably the autoimmune disease, systemic lupus erythematosus. The most frequent cause of a C4 "null" allele, following that of C4 gene copy number variation (CNV), is a non-sense mutation arising from a 2 bp CT insertion into codon 1232 of exon 29. Previous attempts to accurately genotype this polymorphism have not been amenable to high-throughput typing, and have been confounded by failure to account for CNV at this locus, as well as by inability to distinguish between paralogs. We have developed a novel, high-throughput, paralog-specific assay to detect the presence and copy number of this polymorphism. We have genotyped healthy cohorts from the United Kingdom (UK) and Spain. Overall, 30/719 (4.17%) individuals from the UK cohort and 8/449 (1.78%) individuals from the Spanish cohort harboured the CT insertion in a C4A gene. A single Spanish individual possessed a C4B CT insertion. There is weak correlation between the C4 CT insertion and flanking MHC polymorphism. Therefore it is important to note that, as with C4 gene CNV, disease-association due to this variant will be missed by current SNP-based genome-wide association strategies.
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spelling doaj-art-b0cf4e5d017a4d9eb2c63322b90790952025-08-20T03:26:38ZengPublic Library of Science (PLoS)PLoS ONE1932-62032011-01-0168e2212810.1371/journal.pone.0022128Determination of the loss of function complement C4 exon 29 CT insertion using a novel paralog-specific assay in healthy UK and Spanish populations.Lora BotevaIMAGENYee Ling WuJosefina Cortes-HernándezJavier MartinTimothy J VyseMichelle M A FernandoGenetic variants resulting in non-expression of complement C4A and C4B genes are common in healthy European populations and have shown association with a number of diseases, most notably the autoimmune disease, systemic lupus erythematosus. The most frequent cause of a C4 "null" allele, following that of C4 gene copy number variation (CNV), is a non-sense mutation arising from a 2 bp CT insertion into codon 1232 of exon 29. Previous attempts to accurately genotype this polymorphism have not been amenable to high-throughput typing, and have been confounded by failure to account for CNV at this locus, as well as by inability to distinguish between paralogs. We have developed a novel, high-throughput, paralog-specific assay to detect the presence and copy number of this polymorphism. We have genotyped healthy cohorts from the United Kingdom (UK) and Spain. Overall, 30/719 (4.17%) individuals from the UK cohort and 8/449 (1.78%) individuals from the Spanish cohort harboured the CT insertion in a C4A gene. A single Spanish individual possessed a C4B CT insertion. There is weak correlation between the C4 CT insertion and flanking MHC polymorphism. Therefore it is important to note that, as with C4 gene CNV, disease-association due to this variant will be missed by current SNP-based genome-wide association strategies.https://journals.plos.org/plosone/article/file?id=10.1371/journal.pone.0022128&type=printable
spellingShingle Lora Boteva
IMAGEN
Yee Ling Wu
Josefina Cortes-Hernández
Javier Martin
Timothy J Vyse
Michelle M A Fernando
Determination of the loss of function complement C4 exon 29 CT insertion using a novel paralog-specific assay in healthy UK and Spanish populations.
PLoS ONE
title Determination of the loss of function complement C4 exon 29 CT insertion using a novel paralog-specific assay in healthy UK and Spanish populations.
title_full Determination of the loss of function complement C4 exon 29 CT insertion using a novel paralog-specific assay in healthy UK and Spanish populations.
title_fullStr Determination of the loss of function complement C4 exon 29 CT insertion using a novel paralog-specific assay in healthy UK and Spanish populations.
title_full_unstemmed Determination of the loss of function complement C4 exon 29 CT insertion using a novel paralog-specific assay in healthy UK and Spanish populations.
title_short Determination of the loss of function complement C4 exon 29 CT insertion using a novel paralog-specific assay in healthy UK and Spanish populations.
title_sort determination of the loss of function complement c4 exon 29 ct insertion using a novel paralog specific assay in healthy uk and spanish populations
url https://journals.plos.org/plosone/article/file?id=10.1371/journal.pone.0022128&type=printable
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