基因名称(Gene Name)
NFKBIA IKBA MAD3 NFKBI
蛋白名称
NF-kappa-B inhibitor alpha
免疫原
Purified recombinant human IkB-alpha(N-terminus) protein fragments expressed in E.coli.
特异性
This antibody detects endogenous levels of IkB-alpha(N-terminus) and does not cross-react with related proteins.
组成
Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
纯化工艺
The antibody was affinity-purified from mouse ascites by affinity-chromatography using epitope-specific immunogen.
其他名称
I kappa B alpha;I kappa B alpha;I(Kappa)B(alpha);I(Kappa)B(alpha);I-kappa-B-alpha; IkappaBalpha;IkB-alpha;IKBA;IKBA;IKBA_HUMAN;IKBalpha;MAD 3;MAD 3;MAD3;Major histocompatibility complex enhancer binding protein MAD3;Major histocompatibility complex enhancer binding protein MAD3;Major histocompatibility complex enhancer-binding protein MAD3;NF kappa B inhibitor alpha;NF-kappa-B inhibitor alpha;NFKBI;NFKBI;NFKBIA; NFKBIA;Nuclear factor of kappa light chain gene enhancer in B cells;Nuclear factor of kappa light chain gene enhancer in B cells;Nuclear factor of kappa light polypeptide gene enhancer in B cells inhibitor alpha;Nuclear factor of kappa light polypeptide gene enhancer in B cells inhibitor alpha.
背景
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. [provided by RefSeq, Aug 2011],
功能
disease:Defects in NFKBIA are the cause of ectodermal dysplasia anhidrotic with T-cell immunodeficiency autosomal dominant (ADEDAID) [MIM:612132]. Ectodermal dysplasia defines a heterogeneous group of disorders due to abnormal development of two or more ectodermal structures. ADEDAID is an ectodermal dysplasia associated with decreased production of pro-inflammatory cytokines and certain interferons, rendering patients susceptible to infection.,function: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 tranlocate to the nucleus and activate transcription.,induction:Induced in adherent monocytes.,online information:NFKBIA mutation
