A point mutation in NEMO associated with anhidrotic ectodermal dysplasia with immunodeficiency pathology results in destabilization of the oligomer and reduces lipopolysaccharide- and tumor necrosis factor-mediated NF-kappa B activation.
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Intermolecular disulfide bond formation in the NEMO dimer requires Cys54 and Cys347Structural Basis for Recognition of Diubiquitins by NEMONew mechanism of X-linked anhidrotic ectodermal dysplasia with immunodeficiency: impairment of ubiquitin binding despite normal folding of NEMO protein.Dento-craniofacial phenotypes and underlying molecular mechanisms in hypohidrotic ectodermal dysplasia (HED): a review.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.The PP4R1 sub-unit of protein phosphatase PP4 is essential for inhibition of NF-κB by merkel polyomavirus small tumour antigen.The zinc finger of NEMO is a functional ubiquitin-binding domain.Two-sided ubiquitin binding of NF-κB essential modulator (NEMO) zinc finger unveiled by a mutation associated with anhidrotic ectodermal dysplasia with immunodeficiency syndrome.Alterations of the IKBKG locus and diseases: an update and a report of 13 novel mutations.Evidence for M1-Linked Polyubiquitin-Mediated Conformational Change in NEMO.Decreased expression in nuclear factor-κB essential modulator due to a novel splice-site mutation causes X-linked ectodermal dysplasia with immunodeficiency.
P2860
Q24647995-00113140-8A19-447E-B66B-2D81B6F3A431Q27653619-AA9AD256-2E2C-4964-9F85-5D0D6F13D7E8Q35652037-F3DA84B5-614C-4FB0-9C0E-6C96980D7AB3Q37333361-87070A2F-CE66-4EC8-B712-C7A1CD6D5D5FQ38375236-372033CB-87B1-4EBB-9A36-4190ADD7CA81Q38565751-2B743B3A-172C-4755-AEC8-F1B7DE89D741Q38818320-A13A8FCC-FB04-45F9-AF2A-77E2ACF5901EQ39912363-D708563A-0C20-439A-BAE0-E1B7BAF8FA20Q42732317-D71B1106-7BE3-4104-BFF8-CEE14CF910FBQ43785411-06683A70-BCE9-44EA-BE6D-724A42A99B23Q47431945-F510B097-C6DF-4EA6-9B93-8FD7E1ACBE68Q54577574-3F92215D-0EB1-4F11-AB59-50C52EA863E7
P2860
A point mutation in NEMO associated with anhidrotic ectodermal dysplasia with immunodeficiency pathology results in destabilization of the oligomer and reduces lipopolysaccharide- and tumor necrosis factor-mediated NF-kappa B activation.
description
2005 nî lūn-bûn
@nan
2005年の論文
@ja
2005年論文
@yue
2005年論文
@zh-hant
2005年論文
@zh-hk
2005年論文
@zh-mo
2005年論文
@zh-tw
2005年论文
@wuu
2005年论文
@zh
2005年论文
@zh-cn
name
A point mutation in NEMO assoc ...... ediated NF-kappa B activation.
@en
type
label
A point mutation in NEMO assoc ...... ediated NF-kappa B activation.
@en
prefLabel
A point mutation in NEMO assoc ...... ediated NF-kappa B activation.
@en
P2093
P2860
P356
P1476
A point mutation in NEMO assoc ...... ediated NF-kappa B activation.
@en
P2093
Alain Chaffotte
Alain Israël
Emilie Vinolo
Fabrice Agou
Gilles Courtois
Hélène Sebban
Michel Véron
P2860
P304
P356
10.1074/JBC.M510118200
P407
P577
2005-12-26T00:00:00Z