X-linked ectodermal dysplasia with immunodeficiency caused by NEMO mutation: early recognition and diagnosis.
about
Primary immunodeficiencies associated with eosinophiliaIncreased lysis of stem cells but not their differentiated cells by natural killer cells; de-differentiation or reprogramming activates NK cellsNEMO is a key component of NF-κB- and IRF-3-dependent TLR3-mediated immunity to herpes simplex virus.Genetic lessons learned from X-linked Mendelian susceptibility to mycobacterial diseases.Recruitment of A20 by the C-terminal domain of NEMO suppresses NF-κB activation and autoinflammatory disease.The zinc finger domain of IKKγ (NEMO) protein in health and diseaseInsight into IKBKG/NEMO locus: report of new mutations and complex genomic rearrangements leading to incontinentia pigmenti disease.Common dermatologic manifestations of primary immune deficiencies.Rare mendelian primary immunodeficiency diseases associated with impaired NF-κB signalingEDA-ID and IP, two faces of the same coin: how the same IKBKG/NEMO mutation affecting the NF-κB pathway can cause immunodeficiency and/or inflammation.Hematopoietic stem cell transplantation in 29 patients hemizygous for hypomorphic IKBKG / NEMO mutations.Transplantation from a symptomatic carrier sister restores host defenses but does not prevent colitis in NEMO deficiency.The genetic basis of seborrhoeic dermatitis: a review.NEMO syndrome (incontinentia pigmenti) and systemic lupus erythematosus: a new disease association.Successful treatment with infliximab for inflammatory colitis in a patient with X-linked anhidrotic ectodermal dysplasia with immunodeficiency.Challenges in the use of allogeneic hematopoietic SCT for ectodermal dysplasia with immune deficiency.Successful hematopoietic cell transplantation in patients with unique NF-κB essential modulator (NEMO) mutations.
P2860
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P2860
X-linked ectodermal dysplasia with immunodeficiency caused by NEMO mutation: early recognition and diagnosis.
description
2008 nî lūn-bûn
@nan
2008年の論文
@ja
2008年学术文章
@wuu
2008年学术文章
@zh-cn
2008年学术文章
@zh-hans
2008年学术文章
@zh-my
2008年学术文章
@zh-sg
2008年學術文章
@yue
2008年學術文章
@zh
2008年學術文章
@zh-hant
name
X-linked ectodermal dysplasia ...... rly recognition and diagnosis.
@en
X-linked ectodermal dysplasia ...... rly recognition and diagnosis.
@nl
type
label
X-linked ectodermal dysplasia ...... rly recognition and diagnosis.
@en
X-linked ectodermal dysplasia ...... rly recognition and diagnosis.
@nl
prefLabel
X-linked ectodermal dysplasia ...... rly recognition and diagnosis.
@en
X-linked ectodermal dysplasia ...... rly recognition and diagnosis.
@nl
P1433
P1476
X-linked ectodermal dysplasia ...... rly recognition and diagnosis.
@en
P2093
Anthony J Mancini
Leslie P Lawley
P304
P356
10.1001/ARCHDERM.144.3.342
P50
P577
2008-03-01T00:00:00Z