InnateDB: Systems Biology of the Innate Immune Response

Homo sapiens Protein: TSEN34

Summary

InnateDB Protein

IDBP-237606.6

Last Modified

2014-10-13 [Report errors or provide feedback]

Gene Symbol

TSEN34

Protein Name

tRNA splicing endonuclease 34 homolog (S. cerevisiae)

Synonyms

Species

Homo sapiens

Ensembl Protein

ENSP00000379671

InnateDB Gene

IDBG-68287 (TSEN34)

Protein Structure

UniProt Annotation

Function

Constitutes one of the two 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. It probably carries the active site for 3'-splice site cleavage. 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. {ECO:0000269PubMed:15109492}.

Subcellular Localization

Nucleus {ECO:0000269PubMed:15109492}. Nucleus, nucleolus {ECO:0000269PubMed:15109492}. Note=May be transiently localized in the nucleolus.

Disease Associations

Pontocerebellar hypoplasia 2C (PCH2C) [MIM:612390]: A disorder characterized by an abnormally small cerebellum and brainstem, and progressive microcephaly from birth combined with extrapyramidal dyskinesia. Severe chorea occurs and epilepsy is frequent. There are no signs of spinal cord anterior horn cells degeneration. {ECO:0000269PubMed:18711368}. Note=The disease is caused by mutations affecting the gene represented in this entry.

Tissue Specificity

Comments

Interactions

Number of Interactions

This gene and/or its encoded proteins are associated with 18 experimentally validated interaction(s) in this database.

Experimentally validated
Total	18 [view]
Protein-Protein	14 [view]
Protein-DNA	4 [view]
Protein-RNA	0
DNA-DNA	0
RNA-RNA	0
DNA-RNA	0

Gene Ontology

Molecular Function

Accession	GO Term
GO:0000213	tRNA-intron endonuclease activity
GO:0003676	nucleic acid binding
GO:0016829	lyase activity

Biological Process

GO:0000379	tRNA-type intron splice site recognition and cleavage
GO:0006388	tRNA splicing, via endonucleolytic cleavage and ligation
GO:0006397	mRNA processing
GO:0090502	RNA phosphodiester bond hydrolysis, endonucleolytic