Tudor-interacting repair regulator protein (TIRR)

Tudor-interacting repair regulator protein (TIRR, also known as NUDT16L1 or Syndesmos) is a nuclear protein that binds small nucleolar RNA and is a critical regulator of the DNA double-strand break repair pathway. It functions by binding to TP53BP1 (53BP1), masking its Tudor domain and limiting its recruitment to chromatin in the absence of DNA damage. Upon DNA damage, dissociation of TIRR from TP53BP1 unmasks this domain, enabling TP53BP1 participation in DNA repair. TIRR is also involved in RNA binding and has been implicated in the regulation of snoRNA and primary cilia formation. Mutations or misregulation of this protein have been associated with testicular yolk sac tumors and encephalitozoonosis.

Other names

NUDT16L1Nudix hydrolase 16 like 1SyndesmosSDOSNUDT16-like protein 1protein syndesmos