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At least 487 records · Page 27Linked to original sources
PubMed1992

Asparagine-linked sugar chains of plasma membrane glycoproteins from healthy and common variable immunodeficiency B lymphoblastoid cell lines.

Asparagine-linked sugar chains of plasma membrane glycoproteins, which are formed by glycosylation during B cell maturation, were examined with B lymphoblastoid cell lines (LCLs) transformed by Epstein-Barr virus derived from healthy controls and patients with common variable immunodeficiency (CVI). Both two patients with CVI showed hypogammaglobulinemia and impaired B cell functions. LCLs from healthy controls and the patients showed CD19+ and HLA/DR+ in the cell surface and secreted IgM. In both healthy controls and the patients, the main oligosaccharide in asparagine-linked sugar chains of the membrane glycoproteins of LCLs was biantennary sugar chain with bisected GlcNAc (Gal2-GlcNAc2-Man3-GlcNAc-GlcNAc-Fuc-GlcNAcOT). Biantennary sugar chain with an alpha-fucosyl residue linked at the proximal GlcNAc was seen but biantennary sugar chain without an alpha-fucosyl residue at the proximal GlcNAc was little detected in each LCL. There was no difference in quality and quantity of asparagine-linked sugar chains between healthy controls and the patients. These results suggest that glycosylation during B cell maturation may not be impaired in patients with CVI.

Asparagine↗
PubMed1987

Analysis of B cell dysfunction in patients with common variable immunodeficiency by using recombinant interleukin 2.

Recombinant interleukin 2 (RIL2) induces proliferation and differentiation of the Staphylococcus aureus Cowan I (SAC)-activated normal B cells to immunoglobulin (Ig) producting cells. We applied this finding to an analysis of heterogeneity in the differentiation states of B cells in patients with common variable immunodeficiency (CVI). B cells from 5 of 7 patients with CVI were tested for their ability to proliferate under the stimulation of SAC, or SAC and RIL2, and for their differentiation to Ig producing cells in the presence of SAC plus RIL2. The results suggest that the differentiation status of B cells in CVI could be divided into four states based on their responses to SAC and RIL2. In the first state, no B cell proliferation or differentiation was demonstrated in our assays. B cells in the second state showed normal proliferative responses to SAC, but not to SAC plus RIL2, and no differentiation to Ig-secreting cells. The third state showed normal proliferation in response to SAC and SAC plus RIL2, but no differentiation to Ig-secreting cells. The fourth state had a normal proliferation response to SAC and SAC plus RIL2, and normal secretions of IgG and IgM in response to SAC and RIL2. These results show that some B cells in CVI have defects in response to RIL2, and at least in terms of defect of B cells, CVI is heterogeneous.

B-Lymphocytes↗
PubMed1991

Protein kinase C independent restoration of specific immune responsiveness in common variable immunodeficiency.

7,8-Disubstituted guanine ribonucleosides represent a class of B lymphocyte agonists that utilize a protein kinase C-independent signaling pathway. These compounds provide an alternate T helper signal for B cells and enhance antigen-specific humoral responses in the murine model and in an IL-2-dependent human model in vitro. They effectively restore high level immune responses in a variety of murine models of immunodeficiency both in vivo and in vitro. In this study we examined the potential of these compounds to improve antibody responses generated by cultured cells from patients with common variable immunodeficiency (CVI). The inability to mount normal humoral responses to antigen was confirmed in nine patients with diagnosed CVI (CVI: 37 +/- 16, normal 653 +/- 116 plaque-forming cells (PFC)/culture; P less than 0.001). In cultured lymphocytes from eight of the nine patients studied, a normal level or greater responses to nominal antigen could be elicited by antigen in the presence of the immunostimulatory nucleoside 7-methyl-8-oxoguanosine (7m8oGuo). The average response to antigen increased from 37 +/- 16 without nucleoside to 1733 +/- 488 PFC/culture in its presence (P less than 0.002). Restoration of specific immune responses was an antigen-dependent and nucleoside dose-dependent event. Signaling by 7m8oGuo rendered the response to antigen protein kinase C independent in cultures of cells from normal donors as well as from CVI patients. These data substantiate (i) that a non-C-kinase signaling pathway for antigen-dependent differentiation exists, (ii) that this pathway can function normally in B cells from patients with CVI when triggered appropriately, and (iii) that 7,8-disubstituted guanine ribonucleosides can convert a C-kinase-dependent signaling event to a C-kinase-independent signaling event. Substituted guanine ribonucleosides may have potential as immunotherapeutic agents for patients with CVI.

1-(5-Isoquinolinesulfonyl)-2-Methylpiperazine↗
PubMed1995

Expression of surrogate light chain in common variable immunodeficiency.

VpreB and lambda-like genes encode proteins that can associate to form a light-chain-like structure, the surrogate light chain (SLC). VpreB and lambda-like genes are selectively expressed in human progenitor B and precursor B (pre-B) cells. We analyzed the expression of VpreB and lambda-like genes in seven patients with common variable immunodeficiency (CVI). Gene expression was examined in the peripheral blood mononuclear cells (PBMCs) using the polymerase chain reaction. In four CVI patients, two of whom were brothers, the expression of the VpreB gene was detectable in the PBMCs. In the brothers with CVI, the expression of the VpreB gene was also examined in bone marrow cells, where it was found to be expressed in trace amounts. A number of the brothers' bone marrow cells were stained with mu and kappa antibodies using the avidin-biotin complex method. In lambda 5-gene-deleted mice, B cell development in the bone marrow is blocked at the pre-B stage, but the blockade is leaky. We suggest that expression of the SLC is weak in patients with CVI compared with their normal counterparts, and this may be one of the underlying mechanisms of CVI.

Adult↗
PubMed1988

Synthesis of deacetylthymosin beta 11 and its effect on the impaired T-lymphocytes of a uremic patient with common variable immunodeficiency.

The untetracontapeptide corresponding to the entire amino acid sequence of deacetylthymosin beta 11 was synthesized by assembling six peptide fragments via the azide followed by deprotection with 1 M trifluoromethanesulfonic acid-thioanisole in trifluoroacetic acid in the presence of dimethylselenide. The synthetic peptide was tested for its effect on the impaired blastogenic response of phytohemagglutinin-stimulated T-lymphocytes of a uremic patient with common variable immunodeficiency. The synthetic peptide had some restoring activity on the impaired blastogenic response of T-lymphocytes in the one patient tested.

Amino Acid Sequence↗
PubMedDate not supplied

Syntheses and effects of [Glu34]-thymopoietin II fragment 32-45 and its analogs on the impaired T-cell transformation in a patient with common variable immunodeficiency.

[Glu34]-thymopoietin II fragment 32-45 and its three analogs were prepared by substitution of the amino acid residue at position 37. These peptides were synthesized by a conventional solution method, followed by deprotection with 1 M trifluoromethanesulfonic acid-thioanisole in trifluoroacetic acid in the presence of m-cresol. These peptides were tested for their effects on impaired T-cell transformation by phytohemagglutinin in the common variable immunodeficiency. The relative potency of the synthetic [Glu34]-thymopoietin II fragment 32-45 was one-half of that of synthetic thymopoietin II fragment 32-45. Among these tetradecapeptide analogs, one analog in which Val37 was replaced by Ile exhibited a potent activity which was more than that of the synthetic [Glu34]-thymopoietin II fragment 32-45. The relative potencies of Thr37 and Tyr37 analogs were one-third and one-half, respectively of that of the synthetic [Glu34]-thymopoietin II fragment 32-45.

Amino Acid Sequence↗
PubMed1981

Defective cellular immune response in vitro in common variable immunodeficiency.

Mononuclear cells from 39 patients with hypogammaglobulinemia of the common variable type were analyzed for in vitro proliferative response to a panel of cell activators in order to examine the lymphocyte response to mitogens and to study the capacity to generate an immunologically specific secondary response. Patient lymphocyte response to phytohemagglutinin and concanavalin A was found to be significantly lower than that of controls studied in parallel (P less than 0.01), and low response did not correlate with T-lymphocyte number. Response to pokeweed mitogen was significantly lower than that of controls (P less than 0.01), but response to zinc, tested in a few patients, was normal. Strong depressions of patient lymphocyte proliferative responses to Candida albicans, Escherichia coli, and Staphylococcus aureus were observed (P less than 0.01); all of these microbial activators require intact B-cell function for maximum response. Repeated testing of individual patients indicated that poor lymphocyte response could be consistently observed. Examination of change in vitro lymphocyte response during clinical course and disease management showed that a consistent pattern of intrinsic lymphocyte functional deficiency could be demonstrated.

Adolescent↗
PubMed1985

Syntheses and effects of a thymopoietin II fragment and its analogs on the impaired T-cell transformation in a patient with common variable immunodeficiency.

A tetradecapeptide, H-Arg-Lys-Asp-Val-Tyr-Val-Glu-Leu-Tyr-Leu-Gln-Ser-Leu-Thr-OH, corresponding to amino acids 32 to 45 of thymopoietin II and its six analogs by replacing the amino acid residue in position 37, was prepared. These peptides were synthesized using conventional synthesis in solution and were tested for their effect on impaired T-cell transformation by phytohemagglutinin (PHA) in the common variable immunodeficiency. The tetradecapeptide had increasing activity on the T-cell transformation by PHA. Among the tetradecapeptide analogs, several analogs in which Val37 was replaced by Leu or Phe exhibited potent activity which was more than that of the parent peptide fragment. On the other hand, replacement of Val37 by Pro, beta Ala, or sarcosine had no effect at concentrations as high as 3.5 X 10(-4) M. One analog whose Val37 was replaced by Gly showed activity one-third that of the parent peptide fragment. On the basis of these results, the structure-activity relationship for the tetradecapeptide is discussed.

Amino Acid Sequence↗