Medizinische Universität Graz - Research portal
Selected Publication:
Brezinschek, HP; Dörner, T; Monson, NL; Brezinschek, RI; Lipsky, PE.
The influence of CD40-CD154 interactions on the expressed human V(H) repertoire: analysis of V(H) genes expressed by individual B cells of a patient with X-linked hyper-IgM syndrome.
Int Immunol. 2000; 12(6):767-775
Doi: 10.1093/intimm/12.6.767
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- Leading authors Med Uni Graz
- Brezinsek Hans-Peter
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- Abstract:
- Analysis of the V(H)DJ(H) repertoire of peripheral blood IgM(+) B cells from a patient with X-linked hyper-IgM syndrome (X-HIgM) was undertaken to determine whether the distribution of V(H) families in the productive repertoire might be regulated by in vivo CD40-CD154 interactions. The distribution of V(H) genes in the non-productive repertoire of IgM(+) B cells was comparable in X-HIgM and normals. Unlike the normal productive V(H) repertoire, however, in the X-HIgM patient the V(H)4 family was found at almost the same frequency as the V(H)3 family. This reflected a diminution in the positive selection of the V(H)3 family observed in normals and the imposition of positive selection of the V(H)4 family in the X-HIgM patient. Unique among the V(H)3 genes, V(H)3-23/DP-47 was positively selected in both normals and the X-HIgM patient. No major differences in the usage of J(H) or D segments or the complementarity-determining region (CDR) 3 were noted, although the foreshortening of the CDR3 noted in the mutated V(H) rearrangements of normals was absent in the X-HIgM patient. Finally, a minor degree of somatic hypermutation was noted in the X-HIgM patient. These results have suggested that specific influences on the composition of the V(H) repertoire in normals require CD40-CD154 interactions.
- Find related publications in this database (using NLM MeSH Indexing)
- Antigens, CD40 - physiology
- CD40 Ligand - physiology
- Child, Preschool - physiology
- Genes, Immunoglobulin - physiology
- Humans - physiology
- Hypergammaglobulinemia - genetics
- Immunoglobulin Heavy Chains - genetics
- Immunoglobulin M - blood
- Immunoglobulin Variable Region - genetics
- Linkage (Genetics) - genetics
- Male - genetics
- Membrane Glycoproteins - physiology
- Mutation - physiology
- X Chromosome - physiology
- Find related publications in this database (Keywords)
- B lymphocytes
- Ig
- selection
- somatic hypermutation
- VDJ rearrangement