TP53RK binding protein (TPRKB)

TP53RK binding protein (TPRKB) is a protein-coding gene encoding a subunit of the highly conserved EKC/KEOPS protein complex, which is necessary for a key tRNA modification—formation of threonylcarbamoyladenosine (t6A) at position 37 in tRNA molecules that decode ANN codons. TPRKB acts as an allosteric effector within the complex, regulating its t6A modification activity but is not strictly required for the modification itself. It facilitates protein kinase binding and contributes to processes involved in tRNA processing and potentially RNA stability and translation. Mutations in TPRKB cause autosomal recessive Galloway-Mowat syndrome 5, a severe developmental disorder. No specific drugs or clinical interventions are known to act directly via TPRKB.

Other names

CGI-121CGI121EKC/KEOPS complex subunit TPRKBPRPK-binding proteinTP53RK-binding proteinGAMOS5DRWMS10610033G21Rik1810034M08RikMy019LOC100910911LOC103690067mDRWMS1RGD1309786