InnateDB: Systems Biology of the Innate Immune Response

Homo sapiens Protein: TSEN2

Summary

InnateDB Protein

IDBP-364311.5

Last Modified

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

Gene Symbol

TSEN2

Protein Name

tRNA splicing endonuclease 2 homolog (S. cerevisiae)

Synonyms

Species

Homo sapiens

Ensembl Protein

ENSP00000416510

InnateDB Gene

IDBG-19103 (TSEN2)

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. Isoform 1 probably carries the active site for 5'-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. Isoform 2 is responsible for processing a yet unknown RNA substrate. The complex containing isoform 2 is not able to cleave pre-tRNAs properly, although it retains endonucleolytic activity. {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 2B (PCH2B) [MIM:612389]: 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

Isoform 1 and isoform 2 are widely expressed at very low level. {ECO:0000269PubMed:15109492}.

Comments

Interactions

Number of Interactions

This gene and/or its encoded proteins are associated with 15 experimentally validated interaction(s) in this database.

Experimentally validated
Total	15 [view]
Protein-Protein	15 [view]
Protein-DNA	0
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:0006388	tRNA splicing, via endonucleolytic cleavage and ligation
GO:0006397	mRNA processing
GO:0090502	RNA phosphodiester bond hydrolysis, endonucleolytic

Cellular Component

GO:0000214	tRNA-intron endonuclease complex
GO:0005634	nucleus
GO:0005737	cytoplasm
GO:0005813	centrosome

Protein Structure and Domains

PDB ID

InterPro

IPR006677 tRNA intron endonuclease, catalytic domain-like
IPR006678 tRNA intron endonuclease, N-terminal
IPR016589 tRNA-splicing endonuclease, SEN2 subunit

PFAM

PF01974
PF02778

PRINTS

PIRSF

PIRSF011789

SMART

TIGRFAMs

Post-translational Modifications

Modification

Cross-References

SwissProt

Q8NCE0