Specific NEMO mutations impair CD40-mediated c-Rel activation and B cell terminal differentiation.
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SHARPIN is a component of the NF-κB-activating linear ubiquitin chain assembly complexAurora B interacts with NIR-p53, leading to p53 phosphorylation in its DNA-binding domain and subsequent functional suppressionRegulation of aicda expression and AID activity: relevance to somatic hypermutation and class switch DNA recombinationHumoral immunodeficiency : awareness for better supportThe diagnostic approach to monogenic very early onset inflammatory bowel diseaseEducational paper: primary antibody deficienciesPersistent systemic inflammation and atypical enterocolitis in patients with NEMO syndrome.A custom 148 gene-based resequencing chip and the SNP explorer software: new tools to study antibody deficiency.Novel INHAT repressor (NIR) is required for early lymphocyte development.The NEMO mutation creating the most-upstream premature stop codon is hypomorphic because of a reinitiation of translation.Viral double-stranded RNA triggers Ig class switching by activating upper respiratory mucosa B cells through an innate TLR3 pathway involving BAFF.IKBKG (nuclear factor-kappa B essential modulator) mutation can be associated with opportunistic infection without impairing Toll-like receptor function.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.Novel sequencing-based strategies for high-throughput discovery of genetic mutations underlying inherited antibody deficiency disordersPartial immune reconstitution of X-linked hyper IgM syndrome with recombinant CD40 ligandDefective nuclear IKKα function in patients with ectodermal dysplasia with immune deficiency.New mechanism of X-linked anhidrotic ectodermal dysplasia with immunodeficiency: impairment of ubiquitin binding despite normal folding of NEMO protein.c-Rel plays a key role in deficient activation of B cells from a non-X-linked hyper-IgM patient.Impaired dendritic-cell function in ectodermal dysplasia with immune deficiency is linked to defective NEMO ubiquitinationGenetic lessons learned from X-linked Mendelian susceptibility to mycobacterial diseases.Immune responses that adapt the intestinal mucosa to commensal intestinal bacteria.Default in plasma and intestinal IgA responses during acute infection by simian immunodeficiency virus.X-linked susceptibility to mycobacteria is caused by mutations in NEMO impairing CD40-dependent IL-12 production.Recruitment of A20 by the C-terminal domain of NEMO suppresses NF-κB activation and autoinflammatory disease.Immunoglobulin class-switch recombination deficiencies.Impaired priming and activation of the neutrophil NADPH oxidase in patients with IRAK4 or NEMO deficiency.The zinc finger domain of IKKγ (NEMO) protein in health and diseaseMemory B cells: effectors of long-lived immune responses.Unique properties of memory B cells of different isotypes.T cell-B cell interactions in primary immunodeficiencies.NF-κB function in B lymphocytes.DNA damage-dependent NF-κB activation: NEMO turns nuclear signaling inside out.Pathologic Findings in NEMO Deficiency: A Surgical and Autopsy Survey.A novel gain-of-function IKBA mutation underlies ectodermal dysplasia with immunodeficiency and polyendocrinopathy.B cell subsets in healthy children: reference values for evaluation of B cell maturation process in peripheral blood.Decreased expression in nuclear factor-κB essential modulator due to a novel splice-site mutation causes X-linked ectodermal dysplasia with immunodeficiency.
P2860
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P2860
Specific NEMO mutations impair CD40-mediated c-Rel activation and B cell terminal differentiation.
description
article científic
@ca
article scientifique
@fr
articolo scientifico
@it
artigo científico
@pt
bilimsel makale
@tr
scientific article published on December 2004
@en
vedecký článok
@sk
vetenskaplig artikel
@sv
videnskabelig artikel
@da
vědecký článek
@cs
name
Specific NEMO mutations impair ...... cell terminal differentiation.
@en
Specific NEMO mutations impair ...... cell terminal differentiation.
@nl
type
label
Specific NEMO mutations impair ...... cell terminal differentiation.
@en
Specific NEMO mutations impair ...... cell terminal differentiation.
@nl
prefLabel
Specific NEMO mutations impair ...... cell terminal differentiation.
@en
Specific NEMO mutations impair ...... cell terminal differentiation.
@nl
P2093
P2860
P356
P1476
Specific NEMO mutations impair ...... cell terminal differentiation.
@en
P2093
Ashish Jain
Eduardo Lopez-Granados
Gary Means
Jonathan M J Derry
Jordan S Orange
Shuying Liu
Steven Holland
William Brady
P2860
P304
P356
10.1172/JCI21345
P407
P577
2004-12-01T00:00:00Z