TSEN54 : tRNA splicing endonuclease subunit 54
Description
The TSEN54 (tRNA splicing endonuclease subunit 54) is a protein-coding gene located on chromosome 17.
The TSEN54 gene provides instructions for making a subunit of the tRNA splicing endonuclease complex, an enzyme essential for processing various RNA molecules, including transfer RNA (tRNA). tRNA molecules are crucial for protein synthesis, but they require processing before becoming functional. The tRNA splicing endonuclease complex removes introns from tRNAs, enabling their maturation. The complex is also implicated in mRNA processing by potentially cleaving one end of mRNA molecules, allowing for the addition of a poly(A) tail, a signal that stops protein production and protects mRNA from degradation.
TSEN54 is a non-catalytic subunit of the tRNA-splicing endonuclease complex. It plays a crucial role in identifying and cleaving splice sites in pre-tRNA molecules, releasing introns and generating two tRNA half-molecules. The splicing process occurs at specific sites within the pre-tRNA, although the sequences at these sites are not conserved. The complex also participates in mRNA processing by associating with pre-mRNA 3'-end processing factors, suggesting a connection between pre-tRNA splicing and pre-mRNA 3'-end formation. This indicates that TSEN54 and other endonuclease subunits are involved in multiple RNA processing events.
TSEN54 is also known as PCH2A, PCH4, PCH5, SEN54L, sen54.
Associated Diseases
- Pontocerebellar hypoplasia, type 4
- Pontocerebellar hypoplasia type 4
- Pontocerebellar hypoplasia, type 2A
- Pontocerebellar hypoplasia type 2
- Pontocerebellar hypoplasia, type 5
- Pontocerebellar hypoplasia
