publication . Other literature type . Article . 2011

SHARPIN is a component of the NF-κB-activating linear ubiquitin chain assembly complex

Tomoko Nakagawa; Kazuhiro Iwai; Keiji Tanaka; Masaki Nakahara; Masami Taniguchi; Fuminori Tokunaga; Hiroyasu Nakano; Shin-ichi Sakata; Yasushi Saeki;
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  • Published: 30 Mar 2011
  • Publisher: Springer Nature
Abstract
Cpdm (chronic proliferative dermatitis) mice develop chronic dermatitis and an immunodeficiency with increased serum IgM, symptoms that resemble those of patients with X-linked hyper-IgM syndrome and hypohydrotic ectodermal dysplasia (XHM-ED), which is caused by mutations in NEMO (NF-κB essential modulator; also known as IKBKG). Spontaneous null mutations in the Sharpin (SHANK-associated RH domain interacting protein in postsynaptic density) gene are responsible for the cpdm phenotype in mice. SHARPIN shows significant similarity to HOIL-1L (also known as RBCK1), a component of linear ubiquitin chain assembly complex (LUBAC), which induces NF-κB activation throu...
Subjects
free text keywords: Multidisciplinary, NF-κB, chemistry.chemical_compound, chemistry, IKBKG, Ubiquitin, biology.protein, biology, Immunology, Phenotype, Postsynaptic density, Gene, Cell biology, HEK 293 cells, Ratón
Related Organizations
Abstract
Cpdm (chronic proliferative dermatitis) mice develop chronic dermatitis and an immunodeficiency with increased serum IgM, symptoms that resemble those of patients with X-linked hyper-IgM syndrome and hypohydrotic ectodermal dysplasia (XHM-ED), which is caused by mutations in NEMO (NF-κB essential modulator; also known as IKBKG). Spontaneous null mutations in the Sharpin (SHANK-associated RH domain interacting protein in postsynaptic density) gene are responsible for the cpdm phenotype in mice. SHARPIN shows significant similarity to HOIL-1L (also known as RBCK1), a component of linear ubiquitin chain assembly complex (LUBAC), which induces NF-κB activation throu...
Subjects
free text keywords: Multidisciplinary, NF-κB, chemistry.chemical_compound, chemistry, IKBKG, Ubiquitin, biology.protein, biology, Immunology, Phenotype, Postsynaptic density, Gene, Cell biology, HEK 293 cells, Ratón
Related Organizations
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