Summary

The gene codes for a protein, NFKB inhibitor alpha. Mutations may cause ectodermal dysplasia anhidrotic with T-cell immunodeficiency autosomal dominant disease [R].


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 translocate to the nucleus and activate transcription.

Protein names

Recommended name:

NF-kappa-B inhibitor alpha

Alternative name(s):

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

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