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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.
Vinolo E, Sebban H, Chaffotte A, Israël A, Courtois G, Véron M, Agou F. Vinolo E, et al. Among authors: israel a. J Biol Chem. 2006 Mar 10;281(10):6334-48. doi: 10.1074/jbc.M510118200. Epub 2005 Dec 26. J Biol Chem. 2006. PMID: 16379012 Free article.
NEMO trimerizes through its coiled-coil C-terminal domain.
Agou F, Ye F, Goffinont S, Courtois G, Yamaoka S, Israël A, Véron M. Agou F, et al. Among authors: israel a. J Biol Chem. 2002 May 17;277(20):17464-75. doi: 10.1074/jbc.M201964200. Epub 2002 Mar 4. J Biol Chem. 2002. PMID: 11877453 Free article.
Inhibition of NF-kappa B activation by peptides targeting NF-kappa B essential modulator (nemo) oligomerization.
Agou F, Courtois G, Chiaravalli J, Baleux F, Coïc YM, Traincard F, Israël A, Véron M. Agou F, et al. Among authors: israel a. J Biol Chem. 2004 Dec 24;279(52):54248-57. doi: 10.1074/jbc.M406423200. Epub 2004 Oct 5. J Biol Chem. 2004. PMID: 15466857 Free article.
NF-kappa B essential modulator/IKK-gamma (NEMO/IKK-gamma) plays a key role in the activation of the NF-kappa B pathway in response to proinflammatory stimuli. ...The minimal oligomerization domain of this protein consists of two coiled-coil subdomains named Coiled-coil 2 ( …
NF-kappa B essential modulator/IKK-gamma (NEMO/IKK-gamma) plays a key role in the activation of the NF-kappa B pathway in response to …
Osteopetrosis, lymphedema, anhidrotic ectodermal dysplasia, and immunodeficiency in a boy and incontinentia pigmenti in his mother.
Dupuis-Girod S, Corradini N, Hadj-Rabia S, Fournet JC, Faivre L, Le Deist F, Durand P, Döffinger R, Smahi A, Israel A, Courtois G, Brousse N, Blanche S, Munnich A, Fischer A, Casanova JL, Bodemer C. Dupuis-Girod S, et al. Among authors: israel a. Pediatrics. 2002 Jun;109(6):e97. doi: 10.1542/peds.109.6.e97. Pediatrics. 2002. PMID: 12042591
A hypermorphic IkappaBalpha mutation is associated with autosomal dominant anhidrotic ectodermal dysplasia and T cell immunodeficiency.
Courtois G, Smahi A, Reichenbach J, Döffinger R, Cancrini C, Bonnet M, Puel A, Chable-Bessia C, Yamaoka S, Feinberg J, Dupuis-Girod S, Bodemer C, Livadiotti S, Novelli F, Rossi P, Fischer A, Israël A, Munnich A, Le Deist F, Casanova JL. Courtois G, et al. Among authors: israel a. J Clin Invest. 2003 Oct;112(7):1108-15. doi: 10.1172/JCI18714. J Clin Invest. 2003. PMID: 14523047 Free PMC article.
506 results