Recombinant Human tRNA-splicing endonuclease subunit Sen54 (TSEN54)

Uniprot Id

Q7Z6J9

Target Species

Human

Target Name

TSEN54

Target Full Name

tRNA-splicing endonuclease subunit Sen54

Target Function

Non-catalytic subunit of the tRNA-splicing endonuclease complex, a complex responsible for identification and cleavage of the splice sites in pre-tRNA. It cleaves pre-tRNA at the 5' and 3' splice sites to release the intron. The products are an intron and two tRNA half-molecules bearing 2',3' cyclic phosphate and 5'-OH termini. There are no conserved sequences at the splice sites, but the intron is invariably located at the same site in the gene, placing the splice sites an invariant distance from the constant structural features of the tRNA body. The tRNA splicing endonuclease is also involved in mRNA processing via its association with pre-mRNA 3'-end processing factors, establishing a link between pre-tRNA splicing and pre-mRNA 3'-end formation, suggesting that the endonuclease subunits function in multiple RNA-processing events.

Target Involvement

Pontocerebellar hypoplasia 4 (PCH4); Pontocerebellar hypoplasia 2A (PCH2A); Pontocerebellar hypoplasia 5 (PCH5)

Target Subcellular Location

Nucleus. Nucleus, nucleolus.

Target Protein Families

SEN54 family

Target Synonyms

HsSen54; PCH2A; PCH4; sen54; SEN54 homolog; SEN54_HUMAN; SEN54L; tRNA-intron endonuclease SEN54; tRNA-splicing endonuclease subunit SEN54; Tsen54; TSEN54 tRNA splicing endonuclease subunit

Target Background

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.