Abstract:
:Regulatory T (Treg) cells expressing the transcription factor forkhead box P3 (Foxp3) play a requisite role in the maintenance of immunological homeostasis and prevention of peripheral self-tolerance breakdown. Although Foxp3 by itself is neither necessary nor sufficient to specify many aspects of the Treg cell phenotype, its sustained expression in Treg cells is indispensable for their phenotypic stability, metabolic fitness, and regulatory function. In this review, we summarize recent advances in Treg cell biology, with a particular emphasis on the role of Foxp3 as a transcriptional modulator and metabolic gatekeeper essential to an effective immune regulatory response. We discuss these findings in the context of human inborn errors of immune dysregulation, with a focus on FOXP3 mutations, leading to Treg cell deficiency. We also highlight emerging concepts of therapeutic Treg cell reprogramming to restore tolerance in the settings of immune dysregulatory disorders.
journal_name
J Clin Immunoljournal_title
Journal of clinical immunologyauthors
Georgiev P,Charbonnier LM,Chatila TAdoi
10.1007/s10875-019-00684-7subject
Has Abstractpub_date
2019-10-01 00:00:00pages
623-640issue
7eissn
0271-9142issn
1573-2592pii
10.1007/s10875-019-00684-7journal_volume
39pub_type
杂志文章,评审abstract:OBJECTIVE:The aim of this study is to determine when during hematopoiesis Siglec-8 gets expressed, whether it is expressed on hematologic malignancies, and if there are other non-human species that express Siglec-8. METHODS:Siglec-8 mRNA and cell surface expression was monitored during in vitro maturation of human eos...
journal_title:Journal of clinical immunology
pub_type: 杂志文章
doi:10.1007/s10875-011-9589-4
更新日期:2011-12-01 00:00:00
abstract::Sixty patients (16 children, 44 adults) participated in the study aiming at evaluating: (i) IgG levels when switching patients from intravenous IgG (IVIG) infusions in hospital to subcutaneous (SCIG) self-infusions at home using the same cumulative monthly dose, (ii) protections against infections, and (iii) safety of...
journal_title:Journal of clinical immunology
pub_type: 临床试验,杂志文章,多中心研究
doi:10.1007/s10875-006-9002-x
更新日期:2006-03-01 00:00:00
abstract::Antibodies to certain self-antigens are detected in normal individuals as well as in patients with autoimmune diseases. Natural autoantibodies found in normal human sera are thought to act as an immune regulator, a functional controller of specific proteins, or the first-line defense against pathogenic microorganisms....
journal_title:Journal of clinical immunology
pub_type: 杂志文章
doi:10.1023/a:1020599526249
更新日期:1999-05-01 00:00:00
abstract::Intravenous immunoglobin (i.v.IG) is generated from the plasma of more than 10,000 healthy subjects. Originally i.v.IG was employed to supplement the missing Igs in subjects with immune-deficiencies. Later on it was given successfully to patients with autoimmune conditions, in some of which the benefits were confirmed...
journal_title:Journal of clinical immunology
pub_type: 杂志文章,评审
doi:10.1023/b:joci.0000019809.55787.ec
更新日期:2004-03-01 00:00:00
abstract:PURPOSE:Survival of the Leishmania parasite within monocytes hinges on its ability to effectively nullify their microbicidal effector mechanisms. Accordingly, this study aimed to delineate this biological niche in patients with visceral leishmaniasis (VL). METHODS:In monocytes, the redox status, antigen presenting cap...
journal_title:Journal of clinical immunology
pub_type: 杂志文章
doi:10.1007/s10875-014-0115-3
更新日期:2015-01-01 00:00:00
abstract:PURPOSE:Immune thrombocytopenic purpura (ITP) and autoimmune hemolytic anemia (AIHA) are associated in the definition of Evans syndrome (ES). The occurrence of neurological involvement in this population is poorly described and suggests an underlying primary immunodeficiency (PID). We aimed to describe the clinical man...
journal_title:Journal of clinical immunology
pub_type: 杂志文章
doi:10.1007/s10875-019-0594-3
更新日期:2019-02-01 00:00:00
abstract:PURPOSE:To assess the serum profile of factors involved in endothelial, T-cell, and fibroblast interplay in patients with Raynaud's phenomenon (RP) associated with nailfold vodeocapillaroscopy (NVC) scleroderma findings and/or systemic sclerosis (SSc) marker autoantibodies, recently labeled as early SSc patients. METH...
journal_title:Journal of clinical immunology
pub_type: 杂志文章
doi:10.1007/s10875-014-0037-0
更新日期:2014-08-01 00:00:00
abstract:PURPOSE:Mycobacterium leprae exploits complement activation and opsonophagocytosis to infect phagocytes. M-ficolin is encoded by the FCN1 gene and initiates the lectin pathway on monocyte surfaces. We investigated FCN1 promoter polymorphisms that could be responsible for the high interindividual variability of M-ficoli...
journal_title:Journal of clinical immunology
pub_type: 杂志文章
doi:10.1007/s10875-012-9770-4
更新日期:2013-01-01 00:00:00
abstract::Although CpG methylation is thought to be a negative regulator of gene transcription, its relationship with cytokine expression remains unclear. Interleukin (IL)-4 and interferon (IFN)-gamma are major cytokines that affect the differentiation of naïve CD4+ T lymphocytes into the Th1 and Th2 lineage. We used bisulfite ...
journal_title:Journal of clinical immunology
pub_type: 杂志文章
doi:10.1007/s10875-007-9148-1
更新日期:2008-03-01 00:00:00
abstract::The original version of this article unfortunately contained mistake in the following sentence in the Abstract. ...
journal_title:Journal of clinical immunology
pub_type: 已发布勘误
doi:10.1007/s10875-019-00653-0
更新日期:2019-07-01 00:00:00
abstract::Catecholamines have been suggested to be responsible for altered cellular immunity after stress. This study was performed to determine the effects of adrenaline and noradrenaline on lymphocyte subpopulations and NK cell functions. Subjects were given a subcutaneous injection of either NaCl, adrenaline (5 micrograms/kg...
journal_title:Journal of clinical immunology
pub_type: 杂志文章
doi:10.1007/BF00920243
更新日期:1993-09-01 00:00:00
abstract::This phase III, open-label, multi-centre study investigated the efficacy, safety, pharmacokinetics and quality of life impact of Evogam(®), a new chromatographically fractionated 16% subcutaneous immunoglobulin, utilising a 1:1 dose transition ratio from previous immunoglobulin therapy. Thirty-five previously treated ...
journal_title:Journal of clinical immunology
pub_type: 杂志文章,多中心研究
doi:10.1007/s10875-011-9641-4
更新日期:2012-10-01 00:00:00
abstract::Various factors seem to be etiologic in the susceptibility to sinopulmonary infections in ataxia-telangiectasia (A-T) patients, i.e., low serum and salivary IgA, low serum IgG2, and even aspiration of saliva. S. pneumoniae is a common pathogen responsible from pulmonary infections and impaired antibody response to pol...
journal_title:Journal of clinical immunology
pub_type: 杂志文章
doi:10.1023/a:1020599810261
更新日期:1999-09-01 00:00:00
abstract::Severe combined immunodeficiency is a life-threatening primary immune deficiency characterized by low numbers of naïve T cells. Early diagnosis and treatment of this disease decreases mortality. In 2008, Wisconsin began newborn screening of infants for severe combined immunodeficiency and other forms of T-cell lymphop...
journal_title:Journal of clinical immunology
pub_type: 杂志文章
doi:10.1007/s10875-011-9609-4
更新日期:2012-02-01 00:00:00
abstract::Apart from cellular immunity and immunopathology, various cytokines have been implicated in malaria-associated immunosuppression. In this study, serum levels of transforming growth factor-beta (TGF-beta) were determined with an enzyme-linked immunosorbent assay in 37 patients with acute Plasmodium falciparum malaria p...
journal_title:Journal of clinical immunology
pub_type: 杂志文章
doi:10.1007/BF01541734
更新日期:1995-03-01 00:00:00
abstract:BACKGROUND:Wiskott-Aldrich syndrome (WAS) is a rare primary immunodeficiency disease, with an incidence of 4/1,000,000 live male births. In China, an estimated number of 35 babies with WAS are born each year, but likely many remain undiagnosed. OBJECTIVES:The objectives of study were to review the clinical and molecul...
journal_title:Journal of clinical immunology
pub_type: 杂志文章
doi:10.1007/s10875-009-9285-9
更新日期:2009-07-01 00:00:00
abstract:PURPOSE:Home-based subcutaneous immunoglobulin (SCIg) therapy is an alternative to hospital-based intravenous infusions (IVIg). However, SCIg requires patient training and long-term support to ensure proper adherence, optimal efficacy and safety. We evaluated if switching patients to home-based SCIg including an interp...
journal_title:Journal of clinical immunology
pub_type: 杂志文章
doi:10.1007/s10875-016-0288-z
更新日期:2016-07-01 00:00:00
abstract::Chronic enteroviral meningoencephalitis is a well-recognized complication in patients with X-linked agammaglobulinemia (XLA). The majority of published cases refers to its occurrence in patients on no replacement therapy or on only intramuscular immunoglobulin. The advent of intravenous immunoglobulin (IVIg) in the ea...
journal_title:Journal of clinical immunology
pub_type: 杂志文章,评审
doi:10.1007/BF00918150
更新日期:1992-07-01 00:00:00
abstract:PURPOSE:Patients affected by primary immunodeficiency usually undergo a wide range of infections, including reactivation of latent ones. Here we report two cases suffering from late-onset combined immunodeficiency in which ulcerative enteritis due to human Cytomegalovirus caused a life-threatening malabsorption syndrom...
journal_title:Journal of clinical immunology
pub_type: 杂志文章
doi:10.1007/s10875-014-0060-1
更新日期:2014-08-01 00:00:00
abstract::The etiology of immunodeficiency following trauma was investigated. Plasma collected from Fischer rats 1-8 hr following a 40% surface area thermal injury (TI) displays immunosuppressive activity (ISA). Peak ISA (4 hr) exceeded 90% inhibition of Con A3-induced proliferation of normal spleen cells. Splenic macrophage IL...
journal_title:Journal of clinical immunology
pub_type: 杂志文章
doi:10.1007/BF00916942
更新日期:1989-03-01 00:00:00
abstract::Treatment of primary immunodeficiency (PI) is typically initiated with intravenous immunoglobulin (IVIG) loading and then continued with IVIG or subcutaneous IgG (SCIG). This prospective, open-label, multicenter, 6-month study evaluated a new regimen of initiating IgG therapy with SCIG in 18 previously untreated patie...
journal_title:Journal of clinical immunology
pub_type: 临床试验,杂志文章,多中心研究
doi:10.1007/s10875-011-9588-5
更新日期:2011-12-01 00:00:00
abstract:PURPOSE:Systemic lupus erythematous (SLE) is a systemic autoimmune inflammatory disease with both genetic and epigenetic etiologies. Evidence suggests that deregulation of specific genes through epigenetic mechanisms may be a contributing factor to SLE pathology. There is increasing evidence that DNA methyltransferase ...
journal_title:Journal of clinical immunology
pub_type: 杂志文章
doi:10.1007/s10875-012-9803-z
更新日期:2013-02-01 00:00:00
abstract::Rat T lymphoblasts arrested in the G1 phase of the cell cycle by interleukin-2 (IL-2) deprivation can be forced to proceed to the S phase when they are stimulated with IL-2 or the phorbol ester phorbol 12,13-dibutyrate (PDBu). When PDBu is used as a stimulus, extracellular regulated kinase 2 (ERK2) is activated by thr...
journal_title:Journal of clinical immunology
pub_type: 杂志文章
doi:10.1023/a:1027375828134
更新日期:1997-11-01 00:00:00
abstract::The first description of the hybridoma technology in 1975 seemed to hold enormous promise for the treatment of a variety of human disease. The ability to produce monoclonal antibodies led to the availability of large amounts of homogeneous and predictable preparations of antibody. The potential to renew indefinitely a...
journal_title:Journal of clinical immunology
pub_type: 杂志文章,评审
doi:10.1007/BF00918686
更新日期:1990-11-01 00:00:00
abstract::Lymphocytes expressing gammadelta T cell receptors (TCR) constitute an entire system of functionally specialized subsets that have been implicated in the regulation of immune responses, including responses to pathogens and allergens, and in tissue repair. The gammadelta TCRs share structural features with adaptive rec...
journal_title:Journal of clinical immunology
pub_type: 杂志文章,评审
doi:10.1007/s10875-007-9077-z
更新日期:2007-03-01 00:00:00
abstract::Using our gp120/41-expressing, NK cell activity-resistant CEM.NKR cell clones as targets in HIV-1-specific antibody-dependent cellular cytotoxicity (ADCC) assays, we demonstrate here that the serum titers of anti-HIV-1 ADCC antibodies bear a significant (P < 0.05) positive correlation with the peripheral blood CD4+ T ...
journal_title:Journal of clinical immunology
pub_type: 杂志文章
doi:10.1023/a:1011087132180
更新日期:2001-05-01 00:00:00
abstract::Recombinant human erythropoietin (rhEPO) treatment of hemodialyzed (HD) patients normalizes the altered phenotype of CD4(+) lymphocytes and restores the balance of Th1/Th2 cytokines. We decided to test how the presence of rhEPO in cell culture modulates cytokine production of CD4(+) lymphocytes in HD patients with sta...
journal_title:Journal of clinical immunology
pub_type: 杂志文章
doi:10.1007/s10875-012-9835-4
更新日期:2013-04-01 00:00:00
abstract::Total levels of IgM and secretory IgM as well as specific antibodies to poliovirus type I antigen, Escherichia coli O antigens, and beta-lactoglobulin were measured in unstimulated and stimulated saliva as well as nasal secretion using an enzyme-linked immunosorbent assay (ELISA). The levels of these antibodies in IgA...
journal_title:Journal of clinical immunology
pub_type: 杂志文章
doi:10.1007/BF00917328
更新日期:1986-07-01 00:00:00
abstract:PURPOSE:The present study was designed to evaluate the efficacy and safety of a novel, 10% liquid formulation of intravenous immunoglobulin, stabilized with 250 mmol/L L-proline (Privigen), in patients with primary immunodeficiency disease. MATERIALS AND METHODS:Eighty adults and children diagnosed with common variabl...
journal_title:Journal of clinical immunology
pub_type: 杂志文章,多中心研究
doi:10.1007/s10875-008-9231-2
更新日期:2009-01-01 00:00:00
abstract::The staphylococcal enterotoxins have been termed superantigens based on their ability to stimulate polyclonal proliferative responses of murine and human T lymphocytes expressing particular T-cell receptor V beta gene products. Certain of these toxins have been shown both to activate and to induce anergy in reactive T...
journal_title:Journal of clinical immunology
pub_type: 杂志文章
doi:10.1007/BF01540892
更新日期:1995-11-01 00:00:00