EVIDENCE FOR RECESSIVE AND AGAINST DOMINANT INHERITANCE AT THE HLA-LINKED LOCUS IN CELIAC-DISEASE

  • 1 January 1982
    • journal article
    • research article
    • Vol. 34  (2) , 263-277
Abstract
Gluten sensitive enteropathy (GSE) apparently results from the interaction of 2 loci: 1 locus linked to HLA and associated with dominant inheritance, and the other, a non-HLA-linked GSE-associated B-cell alloantigen, exhibiting recessive inheritance. Previous analyses show that a 2-locus, dominant-recessive model is less compatible with the existing population prevalence and observed familial segregation data than is a recessive-recessive 2-locus model. Additional analyses of reported population and familial HLA data are presented that support the recessive mode of inheritance for the HLA-linked disease locus. Reported data from HLA typing of affected sib pairs, the association of GSE with DR3 and DR7 in different populations, and the proportions of different HLA phenotypes and genotypes were compared with expected data derived by 3 different methods. The HLA data analyses consistently reject a dominant mode of inheritance for the presumed HLA-linked disease allele but do not reject a recessive model. The affected sib-pair data also support a recessive model. These analyses are consistent with a previous prediction that the HLA-linked disease allele in GSE is recessively inherited.