The presentation and natural history of immunodeficiency caused by nuclear factor kappaB essential modulator mutation.
about
Primary immunodeficiencies associated with eosinophiliaRole of innate immunity in neonatal infectionNF-kappaB-related genetic diseasesAutoinflammatory periodic fever, immunodeficiency, and thrombocytopenia (PFIT) caused by mutation in actin-regulatory gene WDR1Persistent systemic inflammation and atypical enterocolitis in patients with NEMO syndrome.CC chemokine ligand 3 overcomes the bacteriocidal and phagocytic defect of macrophages and hastens recovery from experimental otitis media in TNF-/- miceTLR signaling and effector functions are intact in XLA neutrophils.Inherited disorders of human Toll-like receptor signaling: immunological implications.2013 IDSA clinical practice guideline for vaccination of the immunocompromised host.The NEMO mutation creating the most-upstream premature stop codon is hypomorphic because of a reinitiation of translation.IKBKG (nuclear factor-kappa B essential modulator) mutation can be associated with opportunistic infection without impairing Toll-like receptor function.Molecular immunity to mycobacteria: knowledge from the mutation and phenotype spectrum analysis of Mendelian susceptibility to mycobacterial diseases.NF-κB Essential Modulator Deficiency Leading to Disseminated Cutaneous Atypical Mycobacteria.Defective lymphoid organogenesis underlies the immune deficiency caused by a heterozygous S32I mutation in IκBα.Infectious diseases in patients with IRAK-4, MyD88, NEMO, or IκBα deficiency.Dendritic cells from humans with hypomorphic mutations in IKBKG/NEMO have impaired mitogen-activated protein kinase activity.NEMO is a key component of NF-κB- and IRF-3-dependent TLR3-mediated immunity to herpes simplex virus.Severe viral infections and primary immunodeficiencies.Impaired dendritic-cell function in ectodermal dysplasia with immune deficiency is linked to defective NEMO ubiquitinationHypohidrotic ectodermal dysplasia and immunodeficiency with coincident NEMO and EDA mutations.Lung manifestations in an autopsy-based series of pulmonary or disseminated nontuberculous mycobacterial diseaseHuman disease resulting from gene mutations that interfere with appropriate nuclear factor-kappaB activation.Toll-like receptor signaling in primary immune deficiencies.Molecular defects in T- and B-cell primary immunodeficiency diseases.Recruitment of A20 by the C-terminal domain of NEMO suppresses NF-κB activation and autoinflammatory disease.Immunity to microbes: lessons from primary immunodeficiencies.Genetic defects of apoptosis and primary immunodeficiency.Unique and shared signaling pathways cooperate to regulate the differentiation of human CD4+ T cells into distinct effector subsets.Hypomorphic nuclear factor-kappaB essential modulator mutation database and reconstitution system identifies phenotypic and immunologic diversity.The zinc finger domain of IKKγ (NEMO) protein in health and diseaseNoncanonical NF-κB signaling is limited by classical NF-κB activity.T cell-B cell interactions in primary immunodeficiencies.Addressing diagnostic challenges in primary immunodeficiencies: laboratory evaluation of Toll-like receptor- and NF-κB-mediated immune responses.The contribution of NF-κB signalling to immune regulation and tolerance.Unraveling the Link Between Ectodermal Disorders and Primary Immunodeficiencies.Persistent nodal histoplasmosis in nuclear factor kappa B essential modulator deficiency: Report of a case and review of infection in primary immunodeficiencies.Human IκBα Gain of Function: a Severe and Syndromic Immunodeficiency.Disseminated BCG infection mimicking metastatic nasopharyngeal carcinoma in an immunodeficient child with a novel hypomorphic NEMO mutation.Pathologic Findings in NEMO Deficiency: A Surgical and Autopsy Survey.IRAK4 and NEMO mutations in otherwise healthy children with recurrent invasive pneumococcal disease.
P2860
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P2860
The presentation and natural history of immunodeficiency caused by nuclear factor kappaB essential modulator mutation.
description
2004 nî lūn-bûn
@nan
2004 թուականի Ապրիլին հրատարակուած գիտական յօդուած
@hyw
2004 թվականի ապրիլին հրատարակված գիտական հոդված
@hy
2004年の論文
@ja
2004年論文
@yue
2004年論文
@zh-hant
2004年論文
@zh-hk
2004年論文
@zh-mo
2004年論文
@zh-tw
2004年论文
@wuu
name
The presentation and natural h ...... essential modulator mutation.
@ast
The presentation and natural h ...... essential modulator mutation.
@en
type
label
The presentation and natural h ...... essential modulator mutation.
@ast
The presentation and natural h ...... essential modulator mutation.
@en
prefLabel
The presentation and natural h ...... essential modulator mutation.
@ast
The presentation and natural h ...... essential modulator mutation.
@en
P2093
P1476
The presentation and natural h ...... essential modulator mutation.
@en
P2093
Ashish Jain
Francisco A Bonilla
Jordan S Orange
Lynda C Schneider
Raif S Geha
Zuhair K Ballas
P304
P356
10.1016/J.JACI.2004.01.762
P407
P577
2004-04-01T00:00:00Z