| Primary Identifier | IPR044882 | Type | Homologous_superfamily |
| Short Name | I2BP1/2_C3HC4-RING_sf |
| description | Interferon regulatory factor 2-binding protein 1 and 2 (IRF-2BP1/2) and their homologue IRF-2BP-like (also known as IRF-2BPL or C14orf4) () are nuclear transcriptional repressor proteins that bind to the C-terminal repression domain of IRF-2 and can inhibit both enhancer-activated and basal transcription. They contain N-terminal zinc finger and C-terminal RING finger domains [, , ]. Mutations in IRF2BP2 are responsible for a Familial Form of Common Variable Immunodeficiency Disorder (CVID), one of the most frequently diagnosed forms of primary immunodeficiency characterised by low quantity of IgG and IgA and poor specific antibody production []. IRF2BPL may play a role inthe development of the central nervous system and in neuronal maintenance [].This superfamily represents the C-terminal C3HC4-type RING finger of IRF2BP1/2 and IRF2BPL []. |
