Automated description from the Alliance of Genome Resources (Release 3.0)
Predicted to contribute to tRNA-intron endonuclease activity. Predicted to be involved in tRNA-type intron splice site recognition and cleavage. Predicted to localize to tRNA-intron endonuclease complex. Human ortholog(s) of this gene implicated in pontocerebellar hypoplasia type 2A; pontocerebellar hypoplasia type 4; and pontocerebellar hypoplasia type 5. Is expressed in cerebellum; retina inner nuclear layer; and retina outer nuclear layer. Orthologous to human TSEN54 (tRNA splicing endonuclease subunit 54).