Literature DB >> 2838527

Premature termination of variable gene rearrangement in B lymphocytes from X-linked agammaglobulinemia.

J Schwaber1, R H Chen.   

Abstract

X-linked agammaglobulinemia (XLA) results from failure of B lymphocyte development. Immature B cells from a patient with XLA were found to produce truncated mu and delta immunoglobulin H chains encoded by D-JH-C (mu delta). The 5' terminal sequence of cDNA encoding the H chains is composed of D-JH with the characteristic GGTTTGAAG/CACTGTG consensus sequence utilized for VH gene rearrangement upstream, and a leader sequence that serves for translation of this intermediate stage of rearrangement. Failure of variable region gene rearrangement may underlie the failure of B lymphoid development in XLA.

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Year:  1988        PMID: 2838527      PMCID: PMC442655          DOI: 10.1172/JCI113550

Source DB:  PubMed          Journal:  J Clin Invest        ISSN: 0021-9738            Impact factor:   14.808


  32 in total

1.  Physical change in cytoplasmic messenger ribonucleoproteins in cells treated with inhibitors of mRNA transcription.

Authors:  G Dreyfuss; S A Adam; Y D Choi
Journal:  Mol Cell Biol       Date:  1984-03       Impact factor: 4.272

2.  A simple and very efficient method for generating cDNA libraries.

Authors:  U Gubler; B J Hoffman
Journal:  Gene       Date:  1983-11       Impact factor: 3.688

3.  The D-JH complex is an intermediate to the complete immunoglobulin heavy-chain V-region gene.

Authors:  Y Yaoita; N Matsunami; C Y Choi; H Sugiyama; T Kishimoto; T Honjo
Journal:  Nucleic Acids Res       Date:  1983-11-11       Impact factor: 16.971

4.  Identification and nucleotide sequence of a diversity DNA segment (D) of immunoglobulin heavy-chain genes.

Authors:  H Sakano; Y Kurosawa; M Weigert; S Tonegawa
Journal:  Nature       Date:  1981-04-16       Impact factor: 49.962

5.  Improved method for cloning human B-cell lines.

Authors:  S Hoch; P H Schur; J Schwaber
Journal:  Cell Immunol       Date:  1982-09-15       Impact factor: 4.868

6.  A single-base change at a splice site in a beta 0-thalassemic gene causes abnormal RNA splicing.

Authors:  R Treisman; N J Proudfoot; M Shander; T Maniatis
Journal:  Cell       Date:  1982-07       Impact factor: 41.582

7.  How signal sequences maintain cleavage specificity.

Authors:  G von Heijne
Journal:  J Mol Biol       Date:  1984-02-25       Impact factor: 5.469

8.  Epstein-Barr virus-induced lymphoblastoid cell lines derived from the peripheral blood of patients with X-linked agammaglobulinemia can secrete IgM.

Authors:  D Levitt; H Ochs; R J Wedgwood
Journal:  J Clin Immunol       Date:  1984-03       Impact factor: 8.317

9.  Early pre-B cells from normal and X-linked agammaglobulinaemia produce C mu without an attached VH region.

Authors:  J Schwaber; H Molgaard; S H Orkin; H J Gould; F S Rosen
Journal:  Nature       Date:  1983 Jul 28-Aug 3       Impact factor: 49.962

10.  Human immunoglobulin D segments encoded in tandem multigenic families.

Authors:  U Siebenlist; J V Ravetch; S Korsmeyer; T Waldmann; P Leder
Journal:  Nature       Date:  1981-12-17       Impact factor: 49.962

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  5 in total

1.  Germ line transcription of the immunoglobulin heavy chain locus directs production of mu chain without VDJ.

Authors:  J Schwaber; B Malone
Journal:  J Clin Invest       Date:  1992-06       Impact factor: 14.808

2.  Nucleotide sequence of an immunoglobulin D-J gene fusion from a B-cell prolymphocytic leukaemia.

Authors:  M A Kennedy; P E Crossen
Journal:  Nucleic Acids Res       Date:  1989-06-26       Impact factor: 16.971

3.  Bone marrow cells in X-linked agammaglobulinemia express pre-B-specific genes (lambda-like and V pre-B) and present immunoglobulin V-D-J gene usage strongly biased to a fetal-like repertoire.

Authors:  M Milili; F Le Deist; G de Saint-Basile; A Fischer; M Fougereau; C Schiff
Journal:  J Clin Invest       Date:  1993-04       Impact factor: 14.808

4.  Correction of the molecular defect in B lymphocytes from X-linked agammaglobulinemia by cell fusion.

Authors:  J Schwaber; N Koenig; J Girard
Journal:  J Clin Invest       Date:  1988-10       Impact factor: 14.808

5.  Clonal diversity in the B cell repertoire of patients with X-linked agammaglobulinemia.

Authors:  R Anker; M E Conley; B A Pollok
Journal:  J Exp Med       Date:  1989-06-01       Impact factor: 14.307

  5 in total

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