N4BP1
Description
The N4BP1 (NEDD4 binding protein 1) is a protein-coding gene located on chromosome 16.
N4BP1 is a potent suppressor of cytokine production and acts as a regulator of innate immune signaling and inflammation. It serves as a key negative regulator of select cytokine and chemokine responses triggered by TRIF-independent Toll-like receptors (TLRs), effectively limiting inflammatory cytokine responses to minor insults. In response to more serious pathogens, N4BP1 is cleaved by CASP8 downstream of TLR3 or TLR4, resulting in its inactivation, which allows the production of inflammatory cytokines. Additionally, N4BP1 acts as a restriction factor against certain viruses, such as HIV-1. It restricts HIV-1 replication by binding to HIV-1 mRNAs and promoting their degradation through its ribonuclease activity. N4BP1 also functions as an inhibitor of the E3 ubiquitin-protein ligase ITCH by interacting with the second WW domain of ITCH. This interaction leads to competition with ITCH's substrates and impairment of substrate ubiquitination.
N4BP1 is also known as -.
Associated Diseases
- common variable immunodeficiency
- isolated agammaglobulinemia
- hyper-IgE recurrent infection syndrome 5, autosomal recessive
- immunodeficiency 72 with autoinflammation
- severe combined immunodeficiency, autosomal recessive, T cell-negative, B cell-negative, NK cell-positive
- severe combined immunodeficiency due to CARD11 deficiency
- autoimmune lymphoproliferative syndrome due to CTLA4 haploinsuffiency
