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MeSH Review


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Disease relevance of Agammaglobulinemia


Psychiatry related information on Agammaglobulinemia


High impact information on Agammaglobulinemia


Chemical compound and disease context of Agammaglobulinemia


Biological context of Agammaglobulinemia


Anatomical context of Agammaglobulinemia


Gene context of Agammaglobulinemia

  • We investigated the in vivo effects of one bolus injection (400 mg/kg) of intravenous immunoglobulin (IVIG) on a number of cytokines, soluble cytokine receptors, and interleukin-1 receptor antagonist (IL-1Ra) in plasma in 12 patients with primary hypogammaglobulinemia [25].
  • Heterozygous truncating mutations in the CXCR4 intracellular tail cause a rare genetic disease known as WHIM syndrome (warts, hypogammaglobulinemia, infections, myelokathexis), whose pathophysiology remains unclear [26].
  • RESULTS: An enhanced production of tumor necrosis factor (TNF)-alpha, TNF-beta, and IL-10 was observed in transient hypogammaglobulinemia; whereas secretion of IL-1, IL-4, and IL-6 was essentially similar in the control and patient groups [27].
  • This pattern of immunoglobulin rearrangements, along with the phenotypic cell surface antigen characteristics (CD19-), suggest that an earlier arrest in B cell development than is characteristic of Bruton's X-linked agammaglobulinemia has occurred in this patient [28].
  • Stability and peptide binding specificity of Btk SH2 domain: molecular basis for X-linked agammaglobulinemia [29].

Analytical, diagnostic and therapeutic context of Agammaglobulinemia


  1. Response to 2-chlorodeoxyadenosine in patients with B-cell chronic lymphocytic leukemia resistant to fludarabine. Juliusson, G., Elmhorn-Rosenborg, A., Liliemark, J. N. Engl. J. Med. (1992) [Pubmed]
  2. Decreased gastrin secretion in patients with late-onset hypogammaglobulinemia. den Hartog, G., van der Meer, J.W., Jansen, J.B., van Furth, R., Lamers, C.B. N. Engl. J. Med. (1988) [Pubmed]
  3. Correction of the molecular defect in B lymphocytes from X-linked agammaglobulinemia by cell fusion. Schwaber, J., Koenig, N., Girard, J. J. Clin. Invest. (1988) [Pubmed]
  4. The metabolism of IgE in patients with immunodeficiency states and neoplastic conditions. Iio, A., Strober, W., Broder, S., Polmar, S.H. J. Clin. Invest. (1977) [Pubmed]
  5. X-linked immunodeficiencies. Conley, M.E. Curr. Opin. Genet. Dev. (1994) [Pubmed]
  6. Bacterial arthritis. Ho, G. Current opinion in rheumatology. (1992) [Pubmed]
  7. Mutations in the mu heavy-chain gene in patients with agammaglobulinemia. Yel, L., Minegishi, Y., Coustan-Smith, E., Buckley, R.H., Trübel, H., Pachman, L.M., Kitchingman, G.R., Campana, D., Rohrer, J., Conley, M.E. N. Engl. J. Med. (1996) [Pubmed]
  8. Structure of the high affinity complex of inositol trisphosphate with a phospholipase C pleckstrin homology domain. Ferguson, K.M., Lemmon, M.A., Schlessinger, J., Sigler, P.B. Cell (1995) [Pubmed]
  9. Deficient expression of a B cell cytoplasmic tyrosine kinase in human X-linked agammaglobulinemia. Tsukada, S., Saffran, D.C., Rawlings, D.J., Parolini, O., Allen, R.C., Klisak, I., Sparkes, R.S., Kubagawa, H., Mohandas, T., Quan, S. Cell (1993) [Pubmed]
  10. Hemophilus influenzae type B disease in children vaccinated with type B polysaccharide vaccine. Granoff, D.M., Shackelford, P.G., Suarez, B.K., Nahm, M.H., Cates, K.L., Murphy, T.V., Karasic, R., Osterholm, M.T., Pandey, J.P., Daum, R.S. N. Engl. J. Med. (1986) [Pubmed]
  11. Modulation of suppressor-cell activity by cimetidine in patients with common variable hypogammaglobulinemia. White, W.B., Ballow, M. N. Engl. J. Med. (1985) [Pubmed]
  12. Pure red cell aplasia and hypogammaglobulinemia associated with Tr-cell chronic lymphocytic leukemia. Nagasawa, T., Abe, T., Nakagawa, T. Blood (1981) [Pubmed]
  13. Tissue culture demyelination by normal human serum. Silberberg, D.H., Manning, M.C., Schreiber, A.D. Ann. Neurol. (1984) [Pubmed]
  14. Development of hypogammaglobulinemia in a patient with systemic lupus erythematosus. Tsokos, G.C., Smith, P.L., Balow, J.E. Am. J. Med. (1986) [Pubmed]
  15. Diphenylhydantoin-induced hypogammaglobulinemia in a patient infected with human immunodeficiency virus. Britigan, B.E. Am. J. Med. (1991) [Pubmed]
  16. Deletion within the Src homology domain 3 of Bruton's tyrosine kinase resulting in X-linked agammaglobulinemia (XLA). Zhu, Q., Zhang, M., Rawlings, D.J., Vihinen, M., Hagemann, T., Saffran, D.C., Kwan, S.P., Nilsson, L., Smith, C.I., Witte, O.N., Chen, S.H., Ochs, H.D. J. Exp. Med. (1994) [Pubmed]
  17. Clinical and molecular analysis of patients with defects in micro heavy chain gene. Lopez Granados, E., Porpiglia, A.S., Hogan, M.B., Matamoros, N., Krasovec, S., Pignata, C., Smith, C.I., Hammarstrom, L., Bjorkander, J., Belohradsky, B.H., Casariego, G.F., Garcia Rodriguez, M.C., Conley, M.E. J. Clin. Invest. (2002) [Pubmed]
  18. Mapping of the X-linked agammaglobulinemia locus by use of restriction fragment-length polymorphism. Kwan, S.P., Kunkel, L., Bruns, G., Wedgwood, R.J., Latt, S., Rosen, F.S. J. Clin. Invest. (1986) [Pubmed]
  19. Phosphorylation of two regulatory tyrosine residues in the activation of Bruton's tyrosine kinase via alternative receptors. Wahl, M.I., Fluckiger, A.C., Kato, R.M., Park, H., Witte, O.N., Rawlings, D.J. Proc. Natl. Acad. Sci. U.S.A. (1997) [Pubmed]
  20. Structural basis for chromosome X-linked agammaglobulinemia: a tyrosine kinase disease. Vihinen, M., Vetrie, D., Maniar, H.S., Ochs, H.D., Zhu, Q., Vorechovský, I., Webster, A.D., Notarangelo, L.D., Nilsson, L., Sowadski, J.M. Proc. Natl. Acad. Sci. U.S.A. (1994) [Pubmed]
  21. Lymphocyte ecto-5'-nucleotidase deficiency in agammaglobulinemia. Edwards, N.L., Magilavy, D.B., Cassidy, J.T., Fox, I.H. Science (1978) [Pubmed]
  22. Ecto-5'-nucleotidase activity in human T cell subsets. Decreased numbers of ecto-5'-nucleotidase positive cells from both OKT4+ and OKT8+ cells in patients with hypogammaglobulinemia. Thompson, L.F., Saxon, A., O'Connor, R.D., Fox, R.I. J. Clin. Invest. (1983) [Pubmed]
  23. Syndrome of neutrophil agranulocytosis, hypogammaglobulinemia, and thymoma. Degos, L., Faille, A., Housset, M., Boumsell, L., Rabian, C., Parames, T. Blood (1982) [Pubmed]
  24. Radiosensitivity of ataxia-telangiectasia, X-linked agammaglobulinemia, and related syndromes using a modified colony survival assay. Huo, Y.K., Wang, Z., Hong, J.H., Chessa, L., McBride, W.H., Perlman, S.L., Gatti, R.A. Cancer Res. (1994) [Pubmed]
  25. Release of cytokines, soluble cytokine receptors, and interleukin-1 receptor antagonist after intravenous immunoglobulin administration in vivo. Aukrust, P., Frøland, S.S., Liabakk, N.B., Müller, F., Nordøy, I., Haug, C., Espevik, T. Blood (1994) [Pubmed]
  26. Altered leukocyte response to CXCL12 in patients with warts hypogammaglobulinemia, infections, myelokathexis (WHIM) syndrome. Gulino, A.V., Moratto, D., Sozzani, S., Cavadini, P., Otero, K., Tassone, L., Imberti, L., Pirovano, S., Notarangelo, L.D., Soresina, R., Mazzolari, E., Nelson, D.L., Notarangelo, L.D., Badolato, R. Blood (2004) [Pubmed]
  27. Cytokine production in transient hypogammaglobulinemia and isolated IgA deficiency. Kowalczyk, D., Mytar, B., Zembala, M. J. Allergy Clin. Immunol. (1997) [Pubmed]
  28. Predominance of sterile immunoglobulin transcripts in a female phenotypically resembling Bruton's agammaglobulinemia. de la Morena, M., Haire, R.N., Ohta, Y., Nelson, R.P., Litman, R.T., Day, N.K., Good, R.A., Litman, G.W. Eur. J. Immunol. (1995) [Pubmed]
  29. Stability and peptide binding specificity of Btk SH2 domain: molecular basis for X-linked agammaglobulinemia. Tzeng, S.R., Pai, M.T., Lung, F.D., Wu, C.W., Roller, P.P., Lei, B., Wei, C.J., Tu, S.C., Chen, S.H., Soong, W.J., Cheng, J.W. Protein Sci. (2000) [Pubmed]
  30. Immunosuppressive treatments for myelodysplastic syndromes. Shimamoto, T., Ohyashiki, K. Leuk. Lymphoma (2003) [Pubmed]
  31. Purification of a major tyrosine kinase from RBL-2H3 cells phosphorylating Fc epsilon RI gamma-cytoplasmic domain and identification as the Btk tyrosine kinase. Price, D.J., Kawakami, Y., Kawakami, T., Rivnay, B. Biochim. Biophys. Acta (1995) [Pubmed]
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