RNF126

Description

The RNF126 (ring finger protein 126) is a protein-coding gene located on chromosome 19.

RNF126 is a human gene that encodes a protein known as RING finger protein 126 or E3 ubiquitin-protein ligase RNF126. It plays a crucial role in various cellular processes, including protein degradation, receptor trafficking, and DNA repair. Its primary function is to attach ubiquitin molecules to target proteins, marking them for degradation by the proteasome. RNF126 participates in a quality control mechanism that removes mislocalized proteins from the secretory pathway, ensuring proper protein folding and function. It also influences the endosomal recycling and degradation of several membrane receptors. Furthermore, RNF126 can regulate DNA repair by modifying the activity of the MRE11 protein. This gene's activity is essential for maintaining cellular homeostasis and preventing the accumulation of damaged or misfolded proteins.

RNF126 acts as an E3 ubiquitin ligase, mediating the ubiquitination of various target proteins. Depending on the associated E2 ligase, it can facilitate 'Lys-48' and 'Lys-63'-linked polyubiquitination of substrates. RNF126 is involved in a quality control process dependent on BAG6, ensuring that mislocalized proteins from the secretory pathway are degraded by the proteasome. This function likely arises from its role in providing ubiquitin ligase activity within the BAG6 complex, leading to the ubiquitination of mislocalized hydrophobic proteins and their subsequent targeting to the proteasome. RNF126 may also play a role in the endosomal recycling of IGF2R, the cation-independent mannose-6-phosphate receptor, as well as the endosomal sorting and degradation of membrane receptors including EGFR, FLT3, MET, and CXCR4, by mediating their ubiquitination. Furthermore, RNF126 may promote cell proliferation by ubiquitinating CDKN1A/p21 and targeting it for degradation. It may also monoubiquitinate AICDA.

RNF126 is also known as -.

Associated Diseases

WES Whole Exome Sequencing