The Wiskott-Aldrich syndrome. A disorder with a possible defect in antigen processing or recognition.

PubWeight™: 2.61‹?› | Rank: Top 1%

🔗 View Article (PMID 4172469)

Published in Lancet on May 18, 1968

Authors

R M Blaese, W Strober, R S Brown, T A Waldmann

Articles citing this

X-linked B-lymphocyte immune defect in CBA/HN mice. I. Studies of the function and composition of spleen cells. J Exp Med (1975) 3.70

Wiskott-Aldrich syndrome, a genetically determined cellular immunologic deficiency: clinical and laboratory responses to therapy with transfer factor. Proc Natl Acad Sci U S A (1970) 2.89

Physical map of the BK virus genome. J Virol (1975) 2.18

Studies of the expression of the Wiskott-Aldrich syndrome protein. J Clin Invest (1996) 1.87

Direct interaction of the Wiskott-Aldrich syndrome protein with the GTPase Cdc42. Proc Natl Acad Sci U S A (1996) 1.62

Familial hypercatabolic hypoproteinemia. A disorder of endogenous catabolism of albumin and immunoglobulin. J Clin Invest (1990) 1.35

Binding of cellular proteins to the regulatory region of BK virus DNA. J Virol (1988) 1.31

The response of leucocytes of agammaglobulinaemia subjects to phythohaemagglutinin and anti-immunoglobulin antiserum. Clin Exp Immunol (1969) 1.29

Linkage of the Wiskott-Aldrich syndrome with polymorphic DNA sequences from the human X chromosome. Proc Natl Acad Sci U S A (1987) 1.23

Preparation of human hyperimmune globulin to Haemophilus influenzae b, Streptococcus pneumoniae, and Neisseria meningitidis. Infect Immun (1984) 1.12

Diagnosis and treatment of gastrointestinal disorders in patients with primary immunodeficiency. Clin Gastroenterol Hepatol (2013) 1.09

Immunoglobulins and transient paraproteins in sera of patients with the Wiskott-Aldrich syndrome: a follow-up study. Clin Exp Immunol (1976) 1.06

Hypercatabolism of IgG, IgA, IgM, and albumin in the Wiskott-Aldrich syndrome. A unique disorder of serum protein metabolism. J Clin Invest (1971) 1.05

Comparison of the serology, transforming ability, and polypeptide composition of human papovaviruses isolated from urine. J Virol (1976) 1.04

Abnormalities of chemotactic lymphokine synthesis and mononuclear leukocyte chemotaxis in Wiskott-Aldrich syndrome. J Clin Invest (1974) 1.02

Antigen-binding lymphocytes in children with immune deficiency. Clin Exp Immunol (1972) 1.02

Intravenous immunoglobulin, splenectomy, and antibiotic prophylaxis in Wiskott-Aldrich syndrome. Arch Dis Child (1996) 0.98

Delayed separation of the umbilical cord, severe widespread infections, and immunodeficiency. Arch Dis Child (1981) 0.95

Phytohaemagglutinin-induced cytotoxic effector lymphocyte function in patients with the Wiskott-Aldrich syndrome (WAS). Clin Exp Immunol (1973) 0.91

The metabolism of IgE in patients with immunodeficiency states and neoplastic conditions. J Clin Invest (1977) 0.84

A case of dys-gamma-globulinemia. Arch Dis Child (1970) 0.83

Defective expression of CD23 and autocrine growth-stimulation in Epstein-Barr virus (EBV)-transformed B cells from patients with Wiskott-Aldrich syndrome (WAS). Clin Exp Immunol (1993) 0.83

Disorders of regulatory T cell function in patients with the Wiskott-Aldrich syndrome. Clin Exp Immunol (1984) 0.83

Specific in vitro antimannan-rich antigen of Candida albicans antibody production by sensitized human blood lymphocytes. J Clin Invest (1983) 0.82

Epstein-Barr-virus-transformed lymphoblastoid cell lines derived from patients with X-linked agammaglobulinaemia and Wiskott-Aldrich syndrome: responses to B cell growth and differentiation factors. Clin Exp Immunol (1989) 0.80

Random monocyte migration: an in vitro correlation with the delayed hypersensitivity skin reaction. Clin Exp Immunol (1979) 0.79

ICON: the early diagnosis of congenital immunodeficiencies. J Clin Immunol (2014) 0.77

VH gene family expression in mice with the xid defect. J Exp Med (1991) 0.76

Rubinstein-Taybi syndrome with humoral and cellular defects: a case report. Arch Dis Child (2000) 0.75

Progressive lymphoid system deterioration: a new familial lymphopenic immunological deficiency disease. Clin Exp Immunol (1970) 0.75

Immune deficiency state in a girl with eczema and low serum IgM. Possible female variant of Wiskott-Aldrich syndrome. Arch Dis Child (1970) 0.75

Apparent impairment of primary antibody response with intact immunological memory. Clin Exp Immunol (1970) 0.75

Articles by these authors

A monoclonal antibody (anti-Tac) reactive with activated and functionally mature human T cells. I. Production of anti-Tac monoclonal antibody and distribution of Tac (+) cells. J Immunol (1981) 10.92

Structure of yeast phenylalanine tRNA at 3 A resolution. Nature (1974) 10.27

New rapid gel sequencing method for RNA. Nature (1977) 9.56

A monoclonal antibody that appears to recognize the receptor for human T-cell growth factor; partial characterization of the receptor. Nature (1982) 9.42

Cell contact-dependent immunosuppression by CD4(+)CD25(+) regulatory T cells is mediated by cell surface-bound transforming growth factor beta. J Exp Med (2001) 8.69

Role of suppressor T cells in pathogenesis of common variable hypogammaglobulinaemia. Lancet (1974) 8.13

Molecular cloning and expression of cDNAs for the human interleukin-2 receptor. Nature (1984) 7.37

Antibodies to interleukin 12 abrogate established experimental colitis in mice. J Exp Med (1995) 6.89

Qualitative analysis of immune function in patients with the acquired immunodeficiency syndrome. Evidence for a selective defect in soluble antigen recognition. N Engl J Med (1985) 6.46

Structure of nucleosome core particles of chromatin. Nature (1977) 6.24

Disparate CD4+ lamina propria (LP) lymphokine secretion profiles in inflammatory bowel disease. Crohn's disease LP cells manifest increased secretion of IFN-gamma, whereas ulcerative colitis LP cells manifest increased secretion of IL-5. J Immunol (1996) 6.21

Metabolism of immunoglobulins. Prog Allergy (1969) 5.74

Local and systemic cytokine responses during experimental human influenza A virus infection. Relation to symptom formation and host defense. J Clin Invest (1998) 5.70

Dominant interfering Fas gene mutations impair apoptosis in a human autoimmune lymphoproliferative syndrome. Cell (1995) 5.64

In vivo gene transfer with retroviral vector-producer cells for treatment of experimental brain tumors. Science (1992) 5.47

Co-adjuvant effects of retinoic acid and IL-15 induce inflammatory immunity to dietary antigens. Nature (2011) 5.41

Cyclosporin A inhibits T-cell growth factor gene expression at the level of mRNA transcription. Proc Natl Acad Sci U S A (1984) 4.90

Clustered arrangement of immunoglobulin lambda constant region genes in man. Nature (1981) 4.87

The primary structure of transcription factor TFIIIA has 12 consecutive repeats. FEBS Lett (1985) 4.73

Demonstration of a non-Tac peptide that binds interleukin 2: a potential participant in a multichain interleukin 2 receptor complex. Proc Natl Acad Sci U S A (1986) 4.61

Structure of yeast phenylalanine transfer RNA at 2.5 A resolution. Proc Natl Acad Sci U S A (1975) 4.57

The gene SCL is expressed during early hematopoiesis and encodes a differentiation-related DNA-binding motif. Proc Natl Acad Sci U S A (1989) 4.22

The crystal structure of EcoRV endonuclease and of its complexes with cognate and non-cognate DNA fragments. EMBO J (1993) 4.11

Local administration of antisense phosphorothioate oligonucleotides to the p65 subunit of NF-kappa B abrogates established experimental colitis in mice. Nat Med (1996) 4.09

T lymphocytes with a normal ADA gene accumulate after transplantation of transduced autologous umbilical cord blood CD34+ cells in ADA-deficient SCID neonates. Nat Med (1998) 3.95

Outcomes of stroke patients in Medicare fee for service and managed care. JAMA (1997) 3.91

Impaired synthesis of polyclonal (non-paraprotein) immunoglobulins by circulating lymphocytes from patients with multiple myeloma Role of suppressor cells. N Engl J Med (1975) 3.81

Interleukin 2 (IL-2) augments transcription of the IL-2 receptor gene. Proc Natl Acad Sci U S A (1985) 3.74

Developmental hierarchy of immunoglobulin gene rearrangements in human leukemic pre-B-cells. Proc Natl Acad Sci U S A (1981) 3.73

A crystallographic study of metal-binding to yeast phenylalanine transfer RNA. J Mol Biol (1977) 3.71

Human immunoglobulin kappa light-chain genes are deleted or rearranged in lambda-producing B cells. Nature (1981) 3.64

Oxazolone colitis: A murine model of T helper cell type 2 colitis treatable with antibodies to interleukin 4. J Exp Med (1998) 3.50

Metabolic properties of IgG subclasses in man. J Clin Invest (1970) 3.48

The metabolism of low density lipoprotein in familial type II hyperlipoproteinemia. J Clin Invest (1972) 3.43

The structure, function, and expression of interleukin-2 receptors on normal and malignant lymphocytes. Science (1986) 3.32

Expression of interleukin 2 receptors on activated human B cells. J Exp Med (1984) 3.27

Protein-losing enteropathy. Gastroenterology (1966) 3.21

T-cell regulation of murine IgA synthesis. J Exp Med (1979) 3.11

Estimating hospital costs. A multiple-output analysis. J Health Econ (1986) 3.09

Predominance of histocompatibility antigen HL-A8 in patients with gluten-sensitive enteropathy. J Clin Invest (1972) 3.09

The role of the kidney in the catabolism of Bence Jones proteins and immunoglobulin fragments. J Exp Med (1967) 3.06

Immunoglobulin-gene rearrangements as unique clonal markers in human lymphoid neoplasms. N Engl J Med (1983) 2.96

Animal models of mucosal inflammation and their relation to human inflammatory bowel disease. Curr Opin Immunol (1999) 2.92

Immunoglobulin gene rearrangement and cell surface antigen expression in acute lymphocytic leukemias of T cell and B cell precursor origins. J Clin Invest (1983) 2.91

Accelerated breakdown of immunoglobulin G (IgG) in myotonic dystrophy: a hereditary error of immunoglobulin catabolism. J Clin Invest (1966) 2.91

The mechanism of intestinal uptake and transcellular transport of IgG in the neonatal rat. J Clin Invest (1972) 2.88

Hereditary renal-cell carcinoma associated with a chromosomal translocation. N Engl J Med (1979) 2.82

IL-2-induced activation-induced cell death is inhibited in IL-15 transgenic mice. Proc Natl Acad Sci U S A (2000) 2.80

The interleukin (IL) 2 receptor beta chain is shared by IL-2 and a cytokine, provisionally designated IL-T, that stimulates T-cell proliferation and the induction of lymphokine-activated killer cells. Proc Natl Acad Sci U S A (1994) 2.79

Resource utilization in liver transplantation: effects of patient characteristics and clinical practice. NIDDK Liver Transplantation Database Group. JAMA (1999) 2.78

Current methods of transfer of young people with Type 1 diabetes to adult services. Diabet Med (2002) 2.76

Characterization of the human receptor for T-cell growth factor. Proc Natl Acad Sci U S A (1983) 2.76

Variable amplification of immunoglobulin lambda light-chain genes in human populations. Nature (1983) 2.75

In situ retroviral-mediated gene transfer for the treatment of brain tumors in rats. Cancer Res (1993) 2.74

Rearrangement and expression of immunoglobulin genes and expression of Tac antigen in hairy cell leukemia. Proc Natl Acad Sci U S A (1983) 2.73

A monoclonal antibody (anti-Tac) reactive with activated and functionally mature human T cells. II. Expression of Tac antigen on activated cytotoxic killer T cells, suppressor cells, and on one of two types of helper T cells. J Immunol (1981) 2.73

Further refinement of the structure of yeast tRNAPhe. J Mol Biol (1978) 2.71

Experimental granulomatous colitis in mice is abrogated by induction of TGF-beta-mediated oral tolerance. J Exp Med (1996) 2.68

The effector cells in human peripheral blood mediating mitogen-induced cellular cytotoxicity and antibody-dependent cellular cytotoxicity. J Immunol (1976) 2.65

Blockade of the interleukin-2 receptor by anti-Tac antibody: inhibition of human lymphocyte activation. J Immunol (1983) 2.65

Survival outcomes following liver transplantation (SOFT) score: a novel method to predict patient survival following liver transplantation. Am J Transplant (2008) 2.63

Degradation of collagen by a human granulocyte collagenolytic system. J Clin Invest (1968) 2.62

NIH conference. Gastrointestinal infections in AIDS. Ann Intern Med (1992) 2.62

NIH conference. New insights into common variable immunodeficiency. Ann Intern Med (1993) 2.52

A lymphokine, provisionally designated interleukin T and produced by a human adult T-cell leukemia line, stimulates T-cell proliferation and the induction of lymphokine-activated killer cells. Proc Natl Acad Sci U S A (1994) 2.51

Ataxia-telangiectasia. Medicine (Baltimore) (1972) 2.50

Predominant role of tumor necrosis factor-alpha in human monocyte IL-10 synthesis. J Immunol (1993) 2.48

The mechanism of appearance of immunoglobulin A in nasal secretions in man. J Clin Invest (1967) 2.45

Specific missense mutations in NEMO result in hyper-IgM syndrome with hypohydrotic ectodermal dysplasia. Nat Immunol (2001) 2.42

Current trends in colorectal cancer: site, incidence, mortality and survival in England and Wales. Clin Oncol (R Coll Radiol) (2001) 2.41

A multiinstitutional survey of the Wiskott-Aldrich syndrome. J Pediatr (1994) 2.39

Therapy of malignant brain tumors by intratumoral implantation of retroviral vector-producing cells. Nat Med (1997) 2.39

Distinct populations of dendritic cells are present in the subepithelial dome and T cell regions of the murine Peyer's patch. J Exp Med (1996) 2.38

Hodgkin's disease. Immunologic, clinical, and histologic features of 50 untreated patients. Ann Intern Med (1967) 2.38

Predominant pathogenic role of tumor necrosis factor in experimental colitis in mice. Eur J Immunol (1997) 2.35

Mucosal immunization with HIV-1 peptide vaccine induces mucosal and systemic cytotoxic T lymphocytes and protective immunity in mice against intrarectal recombinant HIV-vaccinia challenge. Proc Natl Acad Sci U S A (1998) 2.33

The Sézary syndrome: a malignant proliferation of helper T cells. J Clin Invest (1976) 2.32

Blocking the CD40L-CD40 interaction in vivo specifically prevents the priming of T helper 1 cells through the inhibition of interleukin 12 secretion. J Exp Med (1996) 2.30

Dermatitis herpetiformis: the skin and the gut. Ann Intern Med (1980) 2.24

IL-15: a pleiotropic cytokine with diverse receptor/signaling pathways whose expression is controlled at multiple levels. Immunity (1996) 2.23

A humanized antibody that binds to the interleukin 2 receptor. Proc Natl Acad Sci U S A (1989) 2.23

Chromosomal translocation in a human leukemic stem-cell line disrupts the T-cell antigen receptor delta-chain diversity region and results in a previously unreported fusion transcript. Proc Natl Acad Sci U S A (1989) 2.23

Crystallographic and biochemical investigation of the lead(II)-catalyzed hydrolysis of yeast phenylalanine tRNA. Biochemistry (1985) 2.22

The p75 peptide is the receptor for interleukin 2 expressed on large granular lymphocytes and is responsible for the interleukin 2 activation of these cells. Proc Natl Acad Sci U S A (1987) 2.22

Separation of human blood monocytes and lymphocytes on a continuous Percoll gradient. J Immunol Methods (1980) 2.21

Regulation of interleukin 2 receptor expression: effects of phorbol diester, phospholipase C, and reexposure to lectin or antigen. J Immunol (1984) 2.20

An inherited disorder of lymphocyte apoptosis: the autoimmune lymphoproliferative syndrome. Ann Intern Med (1999) 2.20

Dynamic, yet structured: The cell membrane three decades after the Singer-Nicolson model. Proc Natl Acad Sci U S A (2003) 2.20

Mechanisms regulating IgA class-specific immunoglobulin production in murine gut-associated lymphoid tissues. I. T cells derived from Peyer's patches that switch sIgM B cells to sIgA B cells in vitro. J Exp Med (1983) 2.19

The T cell-B cell interaction via OX40-OX40L is necessary for the T cell-dependent humoral immune response. J Exp Med (1996) 2.17

Requirement for IRF-1 in the microenvironment supporting development of natural killer cells. Nature (1998) 2.17

Cross-linking of OX40 ligand, a member of the TNF/NGF cytokine family, induces proliferation and differentiation in murine splenic B cells. Immunity (1995) 2.17

TNBS-colitis. Int Rev Immunol (2000) 2.17