Skip Header

You are using a version of browser that may not display all the features of this website. Please consider upgrading your browser.
Protein

tRNA-splicing endonuclease subunit Sen34

Gene

TSEN34

Organism
Homo sapiens (Human)
Status
Reviewed-Annotation score: -Experimental evidence at protein leveli

Functioni

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.1 Publication

Miscellaneous

Belongs to the leukocyte receptor cluster (LRC) present on 19q13.4.

Catalytic activityi

PretRNA = a 3'-half-tRNA molecule with a 5'-OH end + a 5'-half-tRNA molecule with a 2',3'-cyclic phosphate end + an intron with a 2',3'-cyclic phosphate and a 5'-hydroxyl terminus.

Sites

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Active sitei247By similarity1
Active sitei255By similarity1
Active sitei286By similarity1

GO - Molecular functioni

GO - Biological processi

Keywordsi

Molecular functionLyase
Biological processmRNA processing, tRNA processing

Enzyme and pathway databases

BioCyciMetaCyc:HS10201-MONOMER
ReactomeiR-HSA-6784531 tRNA processing in the nucleus

Names & Taxonomyi

Protein namesi
Recommended name:
tRNA-splicing endonuclease subunit Sen34 (EC:4.6.1.16)
Alternative name(s):
Leukocyte receptor cluster member 5
tRNA-intron endonuclease Sen34
Short name:
HsSen34
Gene namesi
Name:TSEN34
Synonyms:LENG5, SEN34
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
Proteomesi
  • UP000005640 Componenti: Chromosome 19

Organism-specific databases

EuPathDBiHostDB:ENSG00000170892.10
HGNCiHGNC:15506 TSEN34
MIMi608754 gene
neXtProtiNX_Q9BSV6

Subcellular locationi

Extracellular region or secreted Cytosol Plasma membrane Cytoskeleton Lysosome Endosome Peroxisome ER Golgi apparatus Nucleus Mitochondrion Manual annotation Automatic computational assertionGraphics by Christian Stolte; Source: COMPARTMENTS

Keywords - Cellular componenti

Nucleus

Pathology & Biotechi

Involvement in diseasei

Pontocerebellar hypoplasia 2C (PCH2C)1 Publication
The disease is caused by mutations affecting the gene represented in this entry.
Disease descriptionA 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.
See also OMIM:612390
Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_05481158R → W in PCH2C. 1 PublicationCorresponds to variant dbSNP:rs113994150EnsemblClinVar.1

Keywords - Diseasei

Disease mutation, Neurodegeneration, Pontocerebellar hypoplasia

Organism-specific databases

DisGeNETi79042
MalaCardsiTSEN34
MIMi612390 phenotype
OpenTargetsiENSG00000170892
Orphaneti2524 Pontocerebellar hypoplasia type 2
PharmGKBiPA134871088

Polymorphism and mutation databases

BioMutaiTSEN34
DMDMi50401668

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
ChainiPRO_00001094631 – 310tRNA-splicing endonuclease subunit Sen34Add BLAST310

Proteomic databases

EPDiQ9BSV6
MaxQBiQ9BSV6
PaxDbiQ9BSV6
PeptideAtlasiQ9BSV6
PRIDEiQ9BSV6
ProteomicsDBi78929

PTM databases

iPTMnetiQ9BSV6
PhosphoSitePlusiQ9BSV6

Expressioni

Gene expression databases

BgeeiENSG00000170892 Expressed in 224 organ(s), highest expression level in blood
CleanExiHS_TSEN34
ExpressionAtlasiQ9BSV6 baseline and differential
GenevisibleiQ9BSV6 HS

Organism-specific databases

HPAiHPA041111
HPA048208

Interactioni

Subunit structurei

tRNA splicing endonuclease is a heterotetramer composed of TSEN2, TSEN15, TSEN34/LENG5 and TSEN54. tRNA splicing endonuclease complex also contains proteins of the pre-mRNA 3'-end processing machinery such as CLP1, CPSF1, CPSF4 and CSTF2.1 Publication

Protein-protein interaction databases

BioGridi122505, 17 interactors
CORUMiQ9BSV6
IntActiQ9BSV6, 2 interactors
STRINGi9606.ENSP00000305524

Structurei

3D structure databases

ProteinModelPortaliQ9BSV6
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Sequence similaritiesi

Belongs to the tRNA-intron endonuclease family.Curated

Phylogenomic databases

eggNOGiKOG4133 Eukaryota
COG1676 LUCA
GeneTreeiENSGT00390000003912
HOGENOMiHOG000232197
HOVERGENiHBG054846
InParanoidiQ9BSV6
KOiK15323
PhylomeDBiQ9BSV6
TreeFamiTF314631

Family and domain databases

Gene3Di3.40.1350.10, 1 hit
InterProiView protein in InterPro
IPR011856 tRNA_endonuc-like_dom_sf
IPR036167 tRNA_intron_Endo_cat-like_sf
IPR006677 tRNA_intron_Endonuc_cat-like
IPR006676 tRNA_splic
IPR016690 tRNA_splic_SEN34
PfamiView protein in Pfam
PF01974 tRNA_int_endo, 1 hit
PIRSFiPIRSF017250 tRNA_splic_SEN34, 1 hit
SUPFAMiSSF53032 SSF53032, 1 hit
TIGRFAMsiTIGR00324 endA, 1 hit

Sequence (1+)i

Sequence statusi: Complete.

This entry has 1 described isoform and 4 potential isoforms that are computationally mapped.Show allAlign All

Q9BSV6-1 [UniParc]FASTAAdd to basket
« Hide
        10         20         30         40         50
MLVVEVANGR SLVWGAEAVQ ALRERLGVGG RTVGALPRGP RQNSRLGLPL
60 70 80 90 100
LLMPEEARLL AEIGAVTLVS APRPDSRHHS LALTSFKRQQ EESFQEQSAL
110 120 130 140 150
AAEARETRRQ ELLEKITEGQ AAKKQKLEQA SGASSSQEAG SSQAAKEDET
160 170 180 190 200
SDGQASGEQE EAGPSSSQAG PSNGVAPLPR SALLVQLATA RPRPVKARPL
210 220 230 240 250
DWRVQSKDWP HAGRPAHELR YSIYRDLWER GFFLSAAGKF GGDFLVYPGD
260 270 280 290 300
PLRFHAHYIA QCWAPEDTIP LQDLVAAGRL GTSVRKTLLL CSPQPDGKVV
310
YTSLQWASLQ
Length:310
Mass (Da):33,652
Last modified:June 1, 2001 - v1
Checksum:i1C1522D030148EC3
GO

Computationally mapped potential isoform sequencesi

There are 4 potential isoforms mapped to this entry.BLASTAlignShow allAdd to basket
EntryEntry nameProtein names
Gene namesLengthAnnotation
E7EQB3E7EQB3_HUMAN
tRNA-splicing endonuclease subunit ...
TSEN34
315Annotation score:
A9C4B9A9C4B9_HUMAN
tRNA-splicing endonuclease subunit ...
TSEN34
251Annotation score:
B0V3J0B0V3J0_HUMAN
tRNA-splicing endonuclease subunit ...
TSEN34
242Annotation score:
A0A0G2JNK5A0A0G2JNK5_HUMAN
tRNA-splicing endonuclease subunit ...
TSEN34
252Annotation score:

Sequence cautioni

The sequence BAB15284 differs from that shown. Reason: Erroneous termination at position 311. Translated as stop.Curated

Natural variant

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_05481158R → W in PCH2C. 1 PublicationCorresponds to variant dbSNP:rs113994150EnsemblClinVar.1
Natural variantiVAR_061149112L → V1 PublicationCorresponds to variant dbSNP:rs17849378EnsemblClinVar.1

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AK025929 mRNA Translation: BAB15284.1 Sequence problems.
CU457734 Genomic DNA No translation available.
CU151838 Genomic DNA No translation available.
CH471135 Genomic DNA Translation: EAW72203.1
BC004530 mRNA Translation: AAH04530.1
BC020805 mRNA Translation: AAH20805.1
CCDSiCCDS42609.1
RefSeqiNP_001070914.1, NM_001077446.3
NP_001269261.1, NM_001282332.1
NP_076980.2, NM_024075.4
XP_011525596.1, XM_011527294.2
XP_011525597.1, XM_011527295.1
UniGeneiHs.15580

Genome annotation databases

EnsembliENST00000302937; ENSP00000305524; ENSG00000170892
ENST00000396383; ENSP00000379667; ENSG00000170892
ENST00000396388; ENSP00000379671; ENSG00000170892
ENST00000611560; ENSP00000480422; ENSG00000274796
ENST00000611798; ENSP00000480899; ENSG00000278605
ENST00000612236; ENSP00000480503; ENSG00000274129
ENST00000612393; ENSP00000478622; ENSG00000278622
ENST00000613310; ENSP00000477766; ENSG00000274078
ENST00000613712; ENSP00000484865; ENSG00000273896
ENST00000613888; ENSP00000481068; ENSG00000278712
ENST00000614948; ENSP00000478156; ENSG00000278622
ENST00000614984; ENSP00000483266; ENSG00000278712
ENST00000615000; ENSP00000479654; ENSG00000278605
ENST00000615079; ENSP00000484694; ENSG00000278605
ENST00000615900; ENSP00000484964; ENSG00000274078
ENST00000615975; ENSP00000484225; ENSG00000278622
ENST00000616063; ENSP00000480964; ENSG00000274129
ENST00000616209; ENSP00000481374; ENSG00000274672
ENST00000617149; ENSP00000481639; ENSG00000273896
ENST00000617902; ENSP00000484465; ENSG00000275165
ENST00000618135; ENSP00000479576; ENSG00000274129
ENST00000619994; ENSP00000482084; ENSG00000275165
ENST00000622524; ENSP00000483436; ENSG00000274672
ENST00000622538; ENSP00000482527; ENSG00000274796
GeneIDi79042
KEGGihsa:79042
UCSCiuc032icq.1 human

Keywords - Coding sequence diversityi

Polymorphism

Similar proteinsi

Cross-referencesi

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AK025929 mRNA Translation: BAB15284.1 Sequence problems.
CU457734 Genomic DNA No translation available.
CU151838 Genomic DNA No translation available.
CH471135 Genomic DNA Translation: EAW72203.1
BC004530 mRNA Translation: AAH04530.1
BC020805 mRNA Translation: AAH20805.1
CCDSiCCDS42609.1
RefSeqiNP_001070914.1, NM_001077446.3
NP_001269261.1, NM_001282332.1
NP_076980.2, NM_024075.4
XP_011525596.1, XM_011527294.2
XP_011525597.1, XM_011527295.1
UniGeneiHs.15580

3D structure databases

ProteinModelPortaliQ9BSV6
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

BioGridi122505, 17 interactors
CORUMiQ9BSV6
IntActiQ9BSV6, 2 interactors
STRINGi9606.ENSP00000305524

PTM databases

iPTMnetiQ9BSV6
PhosphoSitePlusiQ9BSV6

Polymorphism and mutation databases

BioMutaiTSEN34
DMDMi50401668

Proteomic databases

EPDiQ9BSV6
MaxQBiQ9BSV6
PaxDbiQ9BSV6
PeptideAtlasiQ9BSV6
PRIDEiQ9BSV6
ProteomicsDBi78929

Protocols and materials databases

DNASUi79042
Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENST00000302937; ENSP00000305524; ENSG00000170892
ENST00000396383; ENSP00000379667; ENSG00000170892
ENST00000396388; ENSP00000379671; ENSG00000170892
ENST00000611560; ENSP00000480422; ENSG00000274796
ENST00000611798; ENSP00000480899; ENSG00000278605
ENST00000612236; ENSP00000480503; ENSG00000274129
ENST00000612393; ENSP00000478622; ENSG00000278622
ENST00000613310; ENSP00000477766; ENSG00000274078
ENST00000613712; ENSP00000484865; ENSG00000273896
ENST00000613888; ENSP00000481068; ENSG00000278712
ENST00000614948; ENSP00000478156; ENSG00000278622
ENST00000614984; ENSP00000483266; ENSG00000278712
ENST00000615000; ENSP00000479654; ENSG00000278605
ENST00000615079; ENSP00000484694; ENSG00000278605
ENST00000615900; ENSP00000484964; ENSG00000274078
ENST00000615975; ENSP00000484225; ENSG00000278622
ENST00000616063; ENSP00000480964; ENSG00000274129
ENST00000616209; ENSP00000481374; ENSG00000274672
ENST00000617149; ENSP00000481639; ENSG00000273896
ENST00000617902; ENSP00000484465; ENSG00000275165
ENST00000618135; ENSP00000479576; ENSG00000274129
ENST00000619994; ENSP00000482084; ENSG00000275165
ENST00000622524; ENSP00000483436; ENSG00000274672
ENST00000622538; ENSP00000482527; ENSG00000274796
GeneIDi79042
KEGGihsa:79042
UCSCiuc032icq.1 human

Organism-specific databases

CTDi79042
DisGeNETi79042
EuPathDBiHostDB:ENSG00000170892.10
GeneCardsiTSEN34
HGNCiHGNC:15506 TSEN34
HPAiHPA041111
HPA048208
MalaCardsiTSEN34
MIMi608754 gene
612390 phenotype
neXtProtiNX_Q9BSV6
OpenTargetsiENSG00000170892
Orphaneti2524 Pontocerebellar hypoplasia type 2
PharmGKBiPA134871088
GenAtlasiSearch...

Phylogenomic databases

eggNOGiKOG4133 Eukaryota
COG1676 LUCA
GeneTreeiENSGT00390000003912
HOGENOMiHOG000232197
HOVERGENiHBG054846
InParanoidiQ9BSV6
KOiK15323
PhylomeDBiQ9BSV6
TreeFamiTF314631

Enzyme and pathway databases

BioCyciMetaCyc:HS10201-MONOMER
ReactomeiR-HSA-6784531 tRNA processing in the nucleus

Miscellaneous databases

GeneWikiiTSEN34
GenomeRNAii79042
PROiPR:Q9BSV6
SOURCEiSearch...

Gene expression databases

BgeeiENSG00000170892 Expressed in 224 organ(s), highest expression level in blood
CleanExiHS_TSEN34
ExpressionAtlasiQ9BSV6 baseline and differential
GenevisibleiQ9BSV6 HS

Family and domain databases

Gene3Di3.40.1350.10, 1 hit
InterProiView protein in InterPro
IPR011856 tRNA_endonuc-like_dom_sf
IPR036167 tRNA_intron_Endo_cat-like_sf
IPR006677 tRNA_intron_Endonuc_cat-like
IPR006676 tRNA_splic
IPR016690 tRNA_splic_SEN34
PfamiView protein in Pfam
PF01974 tRNA_int_endo, 1 hit
PIRSFiPIRSF017250 tRNA_splic_SEN34, 1 hit
SUPFAMiSSF53032 SSF53032, 1 hit
TIGRFAMsiTIGR00324 endA, 1 hit
ProtoNetiSearch...

Entry informationi

Entry nameiSEN34_HUMAN
AccessioniPrimary (citable) accession number: Q9BSV6
Secondary accession number(s): A6NNB1
, B0V3J1, Q9BVT1, Q9H6H5
Entry historyiIntegrated into UniProtKB/Swiss-Prot: July 19, 2004
Last sequence update: June 1, 2001
Last modified: September 12, 2018
This is version 141 of the entry and version 1 of the sequence. See complete history.
Entry statusiReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program
DisclaimerAny medical or genetic information present in this entry is provided for research, educational and informational purposes only. It is not in any way intended to be used as a substitute for professional medical advice, diagnosis, treatment or care.

Miscellaneousi

Keywords - Technical termi

Complete proteome, Reference proteome

Documents

  1. Human entries with polymorphisms or disease mutations
    List of human entries with polymorphisms or disease mutations
  2. SIMILARITY comments
    Index of protein domains and families
  3. Human chromosome 19
    Human chromosome 19: entries, gene names and cross-references to MIM
  4. Human polymorphisms and disease mutations
    Index of human polymorphisms and disease mutations
  5. MIM cross-references
    Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot
UniProt is an ELIXIR core data resource
Main funding by: National Institutes of Health

We'd like to inform you that we have updated our Privacy Notice to comply with Europe’s new General Data Protection Regulation (GDPR) that applies since 25 May 2018.

Do not show this banner again