Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UNIPROT:Q00604 (X-linked)
 16,883 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

CBA/N mice, which possess an X-linked immunodeficiency (xid), produce a convincing antibody response to lipopolysaccharide derived from Escherichia coli 0113 (LPS 0113), a thymus-independent antigen. The antibody response produced was shown to be specific for the O-polysaccharide moiety of LPS 0113, rather than lipid A or lipid-A-associated protein. The relevance of this finding to the nature of the genetic defect of xid-mice is discussed.
 ...
PMID:Antibody response of immunodeficient (xid) CBA/N mice to Escherichia coli 0113 lipopolysaccharide, a thymus-independent antigen. 618 86

Evidence is presented that mice with X-linked immunodeficiency (xid) express strong Mlsa,d determinants, a putative marker of the mature subset of B cells. Although young (3-5 wk) (CBA/N X DBA/2)F1 male (xid+) mice stimulated only very weak mixed lymphocyte reactions (MLR) to Mlsa,d determinants, older mice (greater than 7 wk) regularly elicited conspicuous responses, despite being totally unresponsive to TNP-Ficoll. Expression of Mlsa,d determinants by xid+ mice was also detected by the procedure of negative selection in vivo. Thus BALB/c T cells were totally depleted of Mlsa,d reactivity after blood to lymph recirculation through 10-wk old irradiated xid+ (CBA/N X DBA/2)1 male mice. Significantly, a marked (90%) reduction in the anti-Mlsa,d response also occurred with T cell filtration through 3-wk xid+ mice, i.e., mice that elicit only minimal primary MLR; filtration through 3-wk xid- normal female mice led to near-complete (99%) negative selection. Collectively these data indicate either, (a) that xid+ mice contain appreciable numbers of cells with at least some of the properties of mature B cells, or (b) that the expression of Mlsa,d determinants is not restricted to mature B cells. In either case, B cells from xid mice cannot be viewed as a simple model for immature normal B cells.
 ...
PMID:Negative selection in vivo reveals expression of strong Mls determinants in mice with X-linked immunodeficiency. 620 96

Immunodeficiency disorders can be classified on clinical grounds into two broad groups according to whether all features are the result of the immune defect (immunodeficiency syndromes) or whether many, even prominent, features cannot be explained by the immune defect (syndromes with immunodeficiency). X-linked agammaglobulinemia and X-linked chronic granulomatous disease are paradigmatic examples of immunodeficiency syndromes. Despite some overlap (for instance extra-immune symptoms, although minor, are present in several variants of severe combined immunodeficiency and chronic granulomatous disease) immunodeficiency syndromes and syndromes with immunodeficiency are easily distinguishable. Together with the pathogenetic classification of the WHO, the present approach to a clinical classification amplifies the operational concept of immunodeficiency also from a therapeutic point of view.
 ...
PMID:Immunodeficiency and syndromes: a nosographic approach. 621 52

A panel of previously characterized monoclonal antibodies: OKT3, OKT4, OKT8, OKT10, OKT11, OKIa1, OKM2; 3A1, 4F2, UCTH1 and 5/9 were used to evaluate peripheral blood mononuclear cells in patients with severe primary immunodeficiencies: three patients with severe combined immunodeficiency, five with X-linked agammaglobulinemia, 20 with common variable hypogammaglobulinemia, 11 with IgA defect, and one with an unclassified form of T cell defect and hypogammaglobulinemia. Surface markers for T and B cells and in some cases functional assays, were also performed. Our results indicate a heterogeneous pattern in patients with severe combined immunodeficiency: one had peripheral blood mononuclear cells negative with all the monoclonal antibodies used; one had an increase in OKM2+ cells, whereas OKT3+ cells were absent; one had defect and imbalance of immunoregulatory T cell subpopulations. Major imbalances of T cell subsets were not detected in patients with X-linked agamma and IgA defect, whereas in some patients with common variable hypogammaglobulinemia an inversion of the physiological ratio between OKT4+ and OKT8+ cells was consistently detected. In an unclassified case of primary immunodeficiency, almost all peripheral blood mononuclear cells formed rosettes with sheep erythrocytes, but lacked antigens detected by monoclonal antibodies. Based on these observations, possible sites of defects in the T cell differentiation are discussed. We believe that monoclonal antibodies are useful for diagnosis, classification, and monitoring of therapy of primary immunodeficiencies.
 ...
PMID:Monoclonal antibody analysis of T cell subsets in 40 patients with immunodeficiencies. 621 27

Peripheral blood mononuclear cells (PBMC) from 40 patients with a variety of primary immunodeficiency diseases were examined for concanavalin A (Con A) inducible suppressor activity against proliferative response of autologous and allogeneic PBMC to phytohaemagglutinin (PHA). 45% (12/27) of the patients with common variable immunodeficiency and 86% (6/7) of the patients with selective IgA deficiency demonstrated lack of Con A-induced suppressor activity against proliferative response of autologous/allogeneic PBMC. 2 of 4 patients with X-linked agammaglobulin and both patients, each with Wiskott-Aldrich syndrome and ataxia-telangiectasia, also showed deficient suppressor function. This study demonstrates a deficiency of Con A-inducible suppressor-cell activity in a variety of immunodeficiency diseases. Possible underlying mechanisms for this functional defects are discussed.
 ...
PMID:Deficiency of concanavalin A induced suppressor cell activity in patients with primary immunodeficiency disorders. 622 64

CBA/N and CBA/CaHN have a significantly longer latent period than other inbred mouse strains between infection with Moloney murine leukemia virus and the appearance of T cell lymphoma. The genetic characteristics of this resistance have been analyzed in the F1 hybrids of CBA/N and CBA/CaHN with BALB H-2 congenic strains. Sexual phenotype and H-2 haplotype significantly influenced survival in the F1 hybrids of CBA/CaHN with BALB. In the F1 with BALB/cJ and BALB/cAnN (both H-2d), the males survived significantly longer than the females; but in the F1 with BALB.K (H-2k) and BALB.B (H-2b), the survival of males and females was the same. Survival was not prolonged by the recessive X-linked immunodeficiency gene xid or other genes on the CBA/N X-chromosome, because the (CBA/N X BALB/c)F1 male and the reciprocal (BALB/c X CBA/N)F1 male, which does not carry the CBA X-chromosome, were equally resistant. H-2 haplotype did not influence survival among the BALB H-2 congenics, and sex had little effect on the resistance of the CBA and BALB parents. These results demonstrate that a sex-dependent gene linked to H-2 significantly influences the expression of CBA genes for lymphoma resistance in the F1 hybrid with BALB.
 ...
PMID:Sex and H-2 haplotype control the resistance of CBA-BALB hybrids to the induction of T cell lymphoma by Moloney leukemia virus. 633 61

The failure to demonstrate normal humoral and cell-mediated immunity (CMI) in patients diagnosed as SCID is seen to reflect the varied pathogenesis of this syndrome. Two major groups of patients have been described, those with or without an associated absence of the enzyme ADA. The heterogeneity of the syndrome is expressed in variable inheritance patterns (particularly defined X-linked or autosomal recessive modes of inheritance), differing clinical presentations, and significant variability in laboratory findings. Some of this heterogeneity of laboratory findings may in fact be contributed to by the high incidence of infection or engraftment of maternal cells in utero. Common to all, however, is the profound deficiency of functional attributes of humoral and cell-mediated immunity. Insight into the biology of this immunodeficiency has advanced steadily in the last decade. Although initially hypothesized to represent a primary lymphoid stem-cell defect, newer technologies to identify and enumerate lymphocyte subpopulations and precursor lymphocytes have revealed the complexity of the disorder. This complexity may now be attributable to a number of abnormalities in the quantitative and qualitative differentiation of these lymphoid stem cells. Functional differentiation of lymphocytes is the result of a progressive and orderly sequence of events. In SCID, lymphocytes of both lineages may be arrested at specific and identifiable stages of maturation, leading to a deficiency of cell-mediated and humoral immunity. In many patients with SCID, the combined immune deficiency may be linked solely to a failure in the stepwise progression of T-cell differentiation.
 ...
PMID:Diagnosis and classification of severe combined immunodeficiency disease. 636 Feb 47

We have modified a fluorescence-activated cell sorter (FACS) to make three independent immunofluorescence measurements on each cell and used this system to study mouse B-lymphocyte subpopulations. An argon-ion laser (emitting at 488 nm) excites fluorescein- and phycoerythrin-labeled reagents, and a tunable dye laser charged with rhodamine 6G (emitting at 615 nm) excites an allophycocyanin-labeled reagent. We report simultaneous measurements of IgM, IgD, and the recently-defined mouse B lymphocyte antigens BLA-1 and BLA-2 on splenic lymphocytes of CBA/J mice and mice of the congenic strain CBA/N (which have an X-linked immunodeficiency [xid]). These data provide information on relationships among the B-cell populations in CBA/J "normal" mice and the defective CBA/N that could not be derived from one- or two-color immunofluorescent measurements. We believe this is the first use of allophycocyanin as an immunofluorescence label.
 ...
PMID:Three-color immunofluorescence analysis of mouse B-lymphocyte subpopulations. 637 Jun 30

Stimulation of resistance induced by muramyl dipeptide (MDP) and its analog, N alpha-MDP-N epsilon-stearoyl-L-lysine [MDP-Lys(L18)], was examined in experimental salmonellosis in CBA/N defective mice with X-linked immunodeficiency to virulent Salmonella enteritidis no. 11. An injection of either MDP or MDP-Lys(L18) did not induce any effective protection, but repeated injections of MDP-Lys(L18) (100 micrograms per mouse per day for 3 days consecutively) to the mice before bacterial challenge gave some protection. Multiple injections with MDPs once a day for several days consecutively strongly increased bactericidal capacity in the peritoneal cavities and spleens of the mice. Moreover, previous injection of the MDPs could elevate the phagocytic function of the reticuloendothelial system in the defective mice. These results indicate that nonspecific resistance of CBA/N defective mice to salmonella infection can be improved by previous administrations of MDPs.
 ...
PMID:Augmentation of protective and antibacterial activity induced by muramyl dipeptides in CBA/N defective mice with X-linked immunodeficiency for Salmonella enteritidis infection. 637 98

Evidence is presented that B cells from mice with X-linked immunodeficiency (xid) differentiate at a slower rate than normal B cells. This conclusion stems from studies in which (B6 X CBA/J)F1 mice were heavily irradiated (1,000 rads) and reconstituted with a mixture of T-depleted marrow cells taken from (a) nondefective B6 mice (H-2b) and (b) xid CBA/N or nondefective CBA/Ca mice (both H-2k). With transfer of CBA/Ca plus B6 marrow cells, the irradiated recipients become repopulated with B cells derived from both parental marrow sources; except for an early imbalance (probably reflecting Hh resistance), the degree of chimerism remained relatively stable over a period of more than 6 months. Very different results occurred with transfer of a mixture of xid CBA/N and normal B6 marrow. Within the first 2 months after marrow reconstitution, a low but significant proportion of the B cells in both spleen and lymph nodes were of CBA/N origin. Thereafter the proportion of these cells fell progressively, and by 6-9 months virtually all of the B cells were of B6 origin. This gradual decline in CBA/N-derived cells did not apply to other cell types, i.e., T cells or pluripotential stem cells. Analogous results were obtained with transfer of CBA/N vs. CBA/Ca marrow cells into sublethally irradiated (750 rads) (CBA/N X DBA/2)F1 male vs. female mice. For example, CBA/N-marrow derived B cells differentiated effectively and survived for long periods in F1 male mice (xid----xid) but not in F1 female mice (xid----normal). The finding that xid B cells eventually disappear in the presence of normal B cells strengthens the view that xid B cells are an abnormal population not represented in normal mice.
 ...
PMID:Physiology of B cells in mice with X-linked immunodeficiency (xid). III. Disappearance of xid B cells in double bone marrow chimeras. 638 37


<< Previous 1 2 3 4 5 6 7 8 9 10 Next >>