InterPro domain: IPR024336

tRNA-splicing endonucleases ( 3.1.27.9 ) catalyse the endonucleolytic cleavage of pre tRNA at the 5' and 3' splice sites to release the intron and produces two half tRNA molecules bearing 5' hydroxyl and 2', 3'-cyclic phosphate termini [ 1 , 2 ]. The genes encoding these proteins are homologous in eukaryotes and archea. The eukaryotic tRNA-splicing endonucleases are heterotetrameric while the archaeal endonucleases can be split into homodimeric and homotetrameric subgroups.

This entry represents the N-terminal domain of Sen54, a non-catalytic subunit of the tRNA-splicing endonuclease complex. Defects in human Sen54 are a cause of pontocerebellar hypoplasia type 4 [ 3 ].

1. RNA-protein interactions of an archaeal homotetrameric splicing endoribonuclease with an exceptional evolutionary history. EMBO J. 16, 6290-300
2. The yeast tRNA splicing endonuclease: a tetrameric enzyme with two active site subunits homologous to the archaeal tRNA endonucleases. Cell 89, 849-58
3. tRNA splicing endonuclease mutations cause pontocerebellar hypoplasia. Nat. Genet. 40, 1113-8