This gene encodes a subunit of the tRNA splicing endonuclease complex, which catalyzes the removal of introns from precursor tRNAs. The complex is also implicated in pre-mRNA 3-prime end processing. Mutations in this gene result in pontocerebellar hypoplasia type 2.[provided by RefSeq, Oct 2009].
HsSEN54; likely ortholog of H. sapiens SEN54L; SEN54; SEN54 homolog; tRNA-intron endonuclease Sen54; tRNA-splicing endonuclease subunit Sen54; TSEN54 tRNA splicing endonuclease subunit