E3 ubiquitin-protein ligase Ring Finger Protein 113A (RNF113A)

Ring Finger Protein 113A (RNF113A) is a ubiquitously expressed human protein encoded by a single-exon gene on the X chromosome. It contains a conserved RING finger domain and a zinc finger domain, classifying it as a RING-type E3 ubiquitin ligase. RNF113A facilitates the transfer of ubiquitin to target proteins, marking them for degradation via the proteasome or lysosome. It also acts as a spliceosome component, regulating pre-mRNA splicing. RNF113A directly binds and polyubiquitinates CXCR4, downregulating receptor signaling and impacting cellular motility. Mutations in RNF113A cause X-linked trichothiodystrophy, a multisystem disorder. Overexpression of RNF113A is associated with aggressive tumor behavior and poor prognosis in various cancers. RNF113A deficiency heightens cellular susceptibility to oxidative stress and alters NRF2-mediated antioxidant responses. Its critical roles in protein turnover, splicing, and cell fate regulation make RNF113A a promising diagnostic and therapeutic candidate for targeted research

Other names

RNF113ARNF113ZNF183CWC24 (Cwc24 homolog)TTD5 (Trichothiodystrophy 5)Zinc finger protein 183

Drugs indirectly affecting RNF113A would likely modulate its E3 ubiquitin ligase activity, leading to altered ubiquitination and degradation of substrates; specifically, RNF113A mediates ubiquitination of CXCR4, affecting its stability and downstream signaling