NFKBIA IKBA MAD3 NFKBI

NF-kappa-B inhibitor alpha ;
I-kappa-B-alpha ;
IkB-alpha ;
IkappaBalpha ;
Major histocompatibility complex enhancer-binding protein MAD3 ;

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 db,PTM:Phosphorylated; disables inhibition of NF-kappa-B DNA-binding activity.,PTM:Sumoylated; sumoylation requires the presence of the nuclear import signal.,PTM:Ubiquitinated; subsequent to stimulus-dependent phosphorylation on serines.,similarity:Belongs to the NF-kappa-B inhibitor family.,similarity:Contains 5 ANK repeats.,subcellular location:Shuttles between the nucleus and the cytoplasm by a nuclear localization signal (NLS) and a CRM1-dependent nuclear export.,subunit:Interacts with RELA; the interaction requires the nuclear import signal. Interacts with NKIRAS1 and NKIRAS2. Part of a 70-90 kDa complex at least consisting of CHUK, IKBKB, NFKBIA, RELA, IKBKAP and MAP3K14. Interacts with HBV protein X. Interacts with RWDD3; the interaction enhances sumoylation.,

protein import into nucleus, translocation, activation of innate immune response, pattern recognition receptor signaling pathway, toll-like receptor signaling pathway, response to molecule of bacterial origin, activation of immune response, positive regulation of immune system process, immune response-activating signal transduction, innate immune response-activating signal transduction, immune response-regulating signal transduction, regulation of transcription, DNA-dependent, regulation of transcription from RNA polymerase II promoter, protein targeting, protein import into nucleus, intracellular protein transport, nucleocytoplasmic transport, apoptosis, anti-apoptosis, cell surface receptor linked signal transduction, intracellular signaling cascade, protein kinase cascade, I-kappaB kinase/NF-kappaB cascade, cytoplasmic sequestering of NF-kappaB, protein localization, cell death, regulation of Notch signaling pathway, response to bacterium, positive regulation of biosynthetic process, negative regulation of signal transduction, response to organic substance, regulation of specific transcription from RNA polymerase II promoter,positive regulation of specific transcription from RNA polymerase II promoter, positive regulation of macromolecule biosynthetic process, positive regulation of macromolecule metabolic process, positive regulation of gene expression,negative regulation of cell communication, regulation of foam cell differentiation, negative regulation of foam cell differentiation, regulation of cholesterol efflux, positive regulation of cholesterol efflux, regulation of lipid storage,negative regulation of lipid storage, regulation of cell death, programmed cell death, protein transport, death, protein import, positive regulation of cellular biosynthetic process, positive regulation of defense response,lipopolysaccharide-mediated signaling pathway, negative regulation of NF-kappaB transcription factor activity,regulation of cellular protein metabolic process, positive regulation of cellular protein metabolic process, regulation of lipid transport, positive regulation of lipid transport, regulation of sterol transport, positive regulation of sterol transport, regulation of cholesterol transport, positive regulation of cholesterol transport, regulation of intracellular transport, negative regulation of intracellular transport, response to peptidoglycan, response to muramyl dipeptide,response to lipopolysaccharide, maintenance of protein location in cell, regulation of gene-specific transcription,regulation of protein localization, regulation of intracellular protein transport, protein localization in organelle, toll-like receptor 4 signaling pathway, protein localization in nucleus, cellular protein localization, regulation of cell proliferation, regulation of protein import into nucleus, negative regulation of protein import into nucleus, regulation of NF-kappaB import into nucleus, negative regulation of NF-kappaB import into nucleus, regulation of apoptosis,regulation of transcription factor import into nucleus, negative regulation of transcription factor import into nucleus,cytoplasmic sequestering of transcription factor, negative regulation of apoptosis, regulation of programmed cell death, negative regulation of programmed cell death, positive regulation of gene-specific transcription, response to exogenous dsRNA, response to dsRNA, negative regulation of DNA binding, negative regulation of transcription factor activity, negative regulation of molecular function, regulation of innate immune response, positive regulation of innate immune response, establishment of protein localization, maintenance of protein location, regulation of transcription,negative regulation of cell differentiation, regulation of myeloid cell differentiation, negative regulation of myeloid cell differentiation, negative regulation of Notch signaling pathway, positive regulation of transcription, DNA-dependent,positive regulation of nucleobase, nucleoside, nucleotide and nucleic acid metabolic process, positive regulation of transcription, positive regulation of transcription from RNA polymerase II promoter, regulation of nucleocytoplasmic transport, negative regulation of nucleocytoplasmic transport, intracellular transport, positive regulation of response to stimulus, positive regulation of immune response, positive regulation of transport, negative regulation of transport,regulation of transcription factor activity, regulation of binding, negative regulation of binding, regulation of DNA binding, nuclear transport, nuclear import, positive regulation of nitrogen compound metabolic process, cytoplasmic sequestering of protein, regulation of protein transport, negative regulation of protein transport, maintenance of location, positive regulation of protein metabolic process, regulation of RNA metabolic process, positive regulation of RNA metabolic process, maintenance of location in cell, regulation of cellular localization, negative regulation of cell death, regulation of establishment of protein localization, nucleotide-binding oligomerization domain containing signaling pathway, nucleotide-binding oligomerization domain containing 1 signaling pathway, nucleotide-binding oligomerization domain containing 2 signaling pathway, cellular macromolecule localization,

Cytoplasm. Nucleus. Shuttles between the nucleus and the cytoplasm by a nuclear localization signal (NLS) and a CRM1-dependent nuclear export. .