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.
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Authors | H P Brezinschek, T Dörner, N L Monson, R I Brezinschek, P E Lipsky |
Journal | International immunology
(Int Immunol)
Vol. 12
Issue 6
Pg. 767-75
(Jun 2000)
ISSN: 0953-8178 [Print] England |
PMID | 10837404
(Publication Type: Journal Article, Research Support, Non-U.S. Gov't, Research Support, U.S. Gov't, P.H.S.)
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Chemical References |
- CD40 Antigens
- Immunoglobulin Heavy Chains
- Immunoglobulin M
- Immunoglobulin Variable Region
- Membrane Glycoproteins
- CD40 Ligand
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Topics |
- CD40 Antigens
(physiology)
- CD40 Ligand
- Child, Preschool
- Genes, Immunoglobulin
- Genetic Linkage
- Humans
- Hypergammaglobulinemia
(genetics)
- Immunoglobulin Heavy Chains
(genetics)
- Immunoglobulin M
(blood)
- Immunoglobulin Variable Region
(genetics)
- Male
- Membrane Glycoproteins
(physiology)
- Mutation
- X Chromosome
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