IKB alpha est un gène codé par le symbole NFKBIA. Communément appelé aussi: NF-kappa-B inhibitor alpha; I-kappa-B-alpha; IkB-alpha; Major histocompatibility complex enhancer-binding protein MAD3; NFKBIA; IKBA; MAD3; NFKBI. IKB alpha a une masse de 35.61kDa, une longueur d'acide aminé de 317, et est impliqué dans Ectodermal dysplasia and immunodeficiency 2.
Nous proposons 6 IKB alpha kits ELISA pour la détection qualitative ou quantitative de IKB alpha à partir d'échantillons Humain, Souris et Rat.
Informations sur les Gènes et les Protéines
Résumé UniProt
Inhibits the activity of dimeric NF-kappa-B/REL complexes by trapping REL dimers in the cytoplasm through masking of their nuclear localization signals. On cellular stimulation by immune and proinflammatory responses, becomes phosphorylated promoting ubiquitination and degradation, enabling the dimeric RELA to translocate to the nucleus and activate transcription.
Résumé Entrez
This gene encodes a member of the NF-kappa-B inhibitor family, which contain multiple ankrin repeat domains. The encoded protein interacts with REL dimers to inhibit NF-kappa-B/REL complexes which are involved in inflammatory responses. The encoded protein moves between the cytoplasm and the nucleus via a nuclear localization signal and CRM1-mediated nuclear export. Mutations in this gene have been found in ectodermal dysplasia anhidrotic with T-cell immunodeficiency autosomal dominant disease.
Implication dans la maladie
Ectodermal dysplasia and immunodeficiency 2: A form of ectoderma dysplasia, a heterogeneous group of disorders due to abnormal development of two or more ectodermal structures. This form of ectodermal dysplasia is associated with decreased production of pro-inflammatory cytokines and certain interferons, rendering patients susceptible to infection. EDAID2 inheritance is autosomal dominant.
Similitudes de séquence
Belongs to the NF-kappa-B inhibitor family.
Modification post-traductionnelle
Phosphorylated; disables inhibition of NF-kappa-B DNA-binding activity. Phosphorylation at positions 32 and 36 is prerequisite to recognition by UBE2D3 leading to polyubiquitination and subsequent degradation.
Localisation cellulaire
Cytoplasm. Nucleus.
Shuttles between the nucleus and the cytoplasm by a nuclear localization signal (NLS) and a CRM1-dependent nuclear export.
