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Query: UNIPROT:Q00604 (X-linked)
 16,883 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We determined the B cell subpopulations that produce the major cross-reactive idiotype (CRIA) associated with the anti-phenylarsonate (ARS) antibody response of A/J mice. Specifically, we examined the B2 subpopulation found in normal mice which, in H-2b mice, bears the I-Ab-encoded determinant Ia.W39; the B1 subpopulation found in mice expressing the CBA/N X-linked immunodeficiency trait (xid); and the B1 subpopulation found in normal mice after the cytotoxic elimination of B2 cells with anti-Ia. W39 and complement. CRIA is expressed in each of these B cell subpopulations. Antigen plays a selective role in the stimulation of distinct B cell sets. ARS conjugates of keyhole limpet hemocyanin (KLH) can activate both the B1 and B2 subpopulations. In contrast, ARS conjugates of synthetic polypeptides under Ir gene control selectively activate the B2 subpopulation in strains that are genetic responders to the carrier. This leads to the establishment of CRIA dominance where CRIA+ anti-ARS antibody is 70 to 95% of the total anti-arsonate antibody response. This class of antigens fails to activate the B1 cells in either normal or xid mice. We compared the CRIA+ antibody produced by selectively activated B2 cells to that produced by the B1 subpopulation in xid mice. For these comparisons, we used competitive radioimmunoassays that employed polyspecific anti-CRIA antiserum or monoclonal anti-CRIA antibodies specific for distinct idiotopes on the heavy chain of CRIA+ antibody. B2 cells produce a CRIA+ anti-ARS antibody that is idiotopically uniform among individual mice, and that closely approximates the hybridoma protein 36-65 (the heavy chain of 36-65 represents the germ line-encoded sequence of the unique CRIA structural gene (25]. In contrast, the CRIA+ antibody produced by the B1 cell subset of xid mice is idiotopically diverse among individual mice, and differs markedly from the 36-65 hybridoma protein. The extent of diversification found in CRIA+ antibody depends on the B cell subpopulation that produces it.
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PMID:Selective activation by thymus-dependent antigens of distinct B cell subpopulations expressing a major cross-reactive idiotype. 643

Evidence is presented that the in vivo differentiation of B cells expressing X-linked immunodeficiency (xid) is controlled by mature T cells. Normal (C57BL/6 X CBA/J)F1 mice were thymectomized (ATx), heavily irradiated, and reconstituted with CBA/N (xid) or CBA/Ca (nondefective) marrow. In contrast to sham-operated mice, ATx recipients of xid marrow showed an almost total absence of Ig+ B cells in lymph nodes (LN) and thoracic duct lymph at 2 mo post-reconstitution ; B cells were markedly reduced in the spleen in some mice but only moderately in others. Addition of mature T cells soon after marrow reconstitution substantially abrogated the B cell depletion. In control experiments with nondefective B cells, the number of B cells developing in ATx irradiated recipients of normal (xid-) marrow cells was not detectably lower than in sham-operated recipients. These data imply that a subset of T-dependent B cells is either missing in normal mice or present in only very small numbers.
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PMID:Physiology of B cells in mice with X-linked immunodeficiency. II. Influence of the thymus and mature T cells on B cell differentiation. 661 Jul 21

The Wiskott-Aldrich syndrome is an X-linked immunodeficiency disorder consisting of the triad of frequent infections, eczema, and profound thrombocytopenia. We evaluated the effects of splenectomy on hemostatic improvement and subsequent clinical course in 16 patients with the Wiskott-Aldrich syndrome. All 16 had an increase in platelet counts to at least 100,000 per cubic millimeter after splenectomy, with the mean increasing from 19,900 per cubic millimeter preoperatively to 262,700 per cubic millimeter after splenectomy. In addition, platelet size, which is characteristically small in this disease, also became normal. Survival after splenectomy correlated with the prophylactic use of antibiotics. Five of seven patients not taking prophylactic antibiotics died of sepis within 33 months of surgery. The mean survival of the nine patients maintained with prophylactic antibiotics, however, was at least 91.4 months, with six of these patients still alive an average of 11.0 years or more after splenectomy. Thus, splenectomy is a useful therapy for a major cause of morbidity and mortality in this complex syndrome.
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PMID:Splenectomy in the management of the thrombocytopenia of the Wiskott-Aldrich syndrome. 676 87

A new "nude" mouse model was developed by successive crossings and backcrossings between athymic nu/nu mice, on an N:NIH(S) background, and female CBA/N mice that have an X-linked immune defect in B-lymphocyte function. The resulting doubly congenic N:NIH(S(II-nu/nu mice maintained the marked thymic hypoplasia and poor development of hair of the nu/nu mice. In contrast to nu/nu and CBA/N mice, in this new mouse model both T-cell zones of lymph nodes and the spleen were depleted of lymphocytes. Lymphocytic follicles were rare and diminutive; not germinal centers were noted. The nodal cortical and paracortical areas were represented principally by connective tissue, endothelial cells, and macrophages, including giant multinucleated cells. No medullary cords were recognized. The mice with combined immunodeficiency supported the growth of human tumor xenografts and were susceptible to murine viral hepatitis.
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PMID:N:NIH(S)-nu/nu mice with combined immunodeficiency: a new model for human tumor heterotransplantation. 696 28

Our knowledge of juvenile periodontitis is still fragmentary. In 50 years we have advanced from the concept of diffuse atrophy of the alveolar bone (Gottlieb 1923) through the theory of non-inflammatory, degenerative disease of the periodontium (Orban & Weinmann 1942) to the present conception of juvenile periodontitis (Manson & Lehner 1974, Waerhaug 1977a) as a periodontal disease appearing in young individuals with inflammation always present. Only the clinical picture of the disease is quite clear as Baer described it (1971): rapid destruction of the alveolar bone, not commensurate with the local irritants, around more than one permanent tooth in otherwise healthy adolescents. The etiology and etiopathogenesis of juvenile periodontitis have remained unknown. The bacteriological findings of Scransky et al. (1970) and Newman et al. (1974), suggesting some Gram-negative rods as an etiological factor, are still controversial. Neither is the theory of Lehner and his coworkers (1974), that juvenile periodontitis is a selective, cell-mediated immunodeficiency condition, fully accepted. Heredity is an etiologic factor for which there is more evidence. Several authors have found a familial pattern of the disease and it might be either an autosomal, recessive trait (Fourel 1972, Jorgenson et al. 1975) or an X-linked dominant disease (Melnick et al. 1976). These two statements are, however, also controversial. The prevalence rates vary from 0.1% to 17.6%. Although there could be racial variations, the estimated prevalence rates also vary within racial groups, suggesting that there must be great variations in methods and diagnostics. Juvenile periodontitis seems to exist in all racial groups throughout the world and although comparable prevalence figures are difficult to obtain, it seems that the disease is less common in Caucasoid populations and more frequent in India and Africa. The claim of female preponderence requires further investigation.
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PMID:Juvenile periodontitis. 698 66

CBA/N mice have an X-linked immunodeficiency that includes a deficient humoral response to sheep red blood cells (SRBC). In order to study the cellular mechanisms of this deficiency we have examined helper T cell function to SRBC in an adoptive transfer system by using 2 different sources of helper T cells. When thymocytes were used as the source of helper T cell precursors in an adoptive transfer system, CBA/N thymocytes were as effective as CBA/Ca thymocytes in inducing CBA/Ca bone marrow cells to develop into both direct and indirect anti-SRBC plaque-forming cells (PFC). However, when SRBC-primed, irradiated recipient mice were used as the source of helper T cells, primed and irradiated CBA/N recipients developed significantly fewer direct and indirect anti-SRBC PFC than similarly treated CBA/Ca recipients when reconstituted with CBA/Ca bone marrow cells and challenged with SRBC. This difference in radioresistant helper T cell function was also observed when primed, irradiated (CBA/N X DBA/2)F1 defective male and nondefective female mice were used as recipients of F1 female bone marrow cells and SRBC, confirming that this defect is a part of the X-linked CBA/N immunodeficiency. This deficiency in radioresistant helper T cell function in CBA/N mice does not appear to be due either to suppressor T cell function, or to a negative effect of the CBA/N environment on either B cell maturation, T cell-B cell cooperation, or helper T cell function. We conclude that antigen-primed helper T cell function in CBA/N mice is radiosensitive. Possible reasons for this are evaluated and discussed.
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PMID:Antigen-primed helper T cell function in CBA/N mice is radiosensitive. 701 24

The IgG subclass distribution of anti-sheep red blood cell (SRBC) plaque-forming cells (PFC) in CBA/N, CBA/Ca, and (CBA/N X CBA/Ca)F1 male and female mice was examined. The results show that the X-linked immunodeficiency of CBA/N mice affects the development of anti-SRBC PFC of all IgG subclasses tested.
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PMID:IgG subclass distribution of anti-sheep red blood cell plaque-forming cells in mice with the CBA/N defect. 703 64

Anti-phosphocholine (PC) antibody mediated protection against many strains of Streptococcus pneumoniae, and hybridoma anti-PC antibodies protected mice from fatal infections with types 1 and 3 S. pneumoniae. Live types 1, 3, 5, 6A, and 19F S. pneumoniae had similar amounts of surface PC accessible to antibody. Furthermore, mice expressing the X-linked immunodeficiency (xid) of the CBA/N strain were found to be more susceptible to infection with S. pneumoniae of types 3, 6A, and 19F than were immunologically normal mice. The only exception to these results was with the type 5 strain, which was highly virulent for both xid and normal mice. In addition, we were unable to protect mice against infection with the type 5 strain by using anti-PC antibody.
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PMID:Protection of mice from infection with Streptococcus pneumoniae by anti-phosphocholine antibody. 707 92

Immunodeficiency syndromes in childhood are partly congenital and due to metabolic errors. A majority are inherited, and from this group examples are presented of autosomal-recessive, X-linked, and dominant transmission. Reliable carrier identification and prenatal diagnosis are possible in a minority of these diseases, but clinical immunologists hope that new techniques will soon offer these possibilities to all families with hereditary forms of IDS.
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PMID:Genetics of immunodeficiency diseases. 716 27

We undertook clinical, immunologic, and endocrinologic studies of a family in which two brothers and their two maternal uncles had a similar disorder characterized by hypogammaglobulinemia and isolated growth hormone deficiency. Recurrent sinopulmonary infections were a prominent feature in two patients. All patients had short stature and retarded bone age during childhood, and the adults had delayed onset of puberty. The immunodeficiency was characterized by absent specific antibody production in vivo and impaired immunoglobulin production in vitro. Three of the four patients lacked circulating B lymphocytes, even though tonsils were present in those patients. All patients had deficient growth hormone responses to insulin and arginine or levodopa. These patients have an X-linked recessive disorder, but their immunodeficiency differs from the X-linked immune disorders in the World Health Organization classification; their X-linked pattern of growth hormone deficiency, without other endocrine abnormality, is also unique.
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PMID:X-linked hypogammaglobulinemia and isolated growth hormone deficiency. 718 77


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