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EC Tree
IUBMB Comments The enzyme catalyses the final stage in the maturation of tRNA molecules.
The taxonomic range for the selected organisms is: Homo sapiens The expected taxonomic range for this enzyme is: Eukaryota, Archaea, Bacteria
Reaction Schemes
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
Synonyms
homing endonuclease, i-tevi, splicing endonuclease, trna splicing endonuclease, hspc117, intron-encoded endonuclease, hclp1, trna-splicing endonuclease, af endonuclease, rna splicing endonuclease,
more
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HEAB
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human homologue to a hypothetical Caenorhabditis elegans ATP/GTP-binding protein
nuclease, transfer ribonucleate intron endoribo-
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splicing endonuclease
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transfer ribonucleate intron endoribonuclease
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transfer splicing endonuclease
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tRNA intron endonuclease
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tRNA splicing endonuclease
tRNA-intron endonuclease
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tRNA-splicing endonuclease
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mutations cause pontocerebellar hypoplasia (Homo sapiens)
tRNATRPintron endonuclease
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tRNA splicing endonuclease
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tRNA splicing endonuclease
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endoribonuclease reaction
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pretRNA lyase (intron-removing; cyclic-2',3'-phosphate-forming)
The enzyme catalyses the final stage in the maturation of tRNA molecules.
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3'-phospho-double stranded-RNA
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?
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
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siRNA + H2O
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RNA kinase responsible for the phosphorylation at the 5' position of tRNA 3' exons
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tRNA + H2O
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enzymatically active component of the tRNA splicing endonuclease
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tRNA precursor
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
tRNA precursor
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
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tRNA precursor
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
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differences in distance and relative helical orientation of the splice sites in plant pre-tRNAs versus pre-tRNAs from other organisms are tolerated by the vertebrate splicing endonuclease, but not at all by the plant enzyme
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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
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?
siRNA + H2O
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RNA kinase responsible for the phosphorylation at the 5' position of tRNA 3' exons
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?
tRNA precursor
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
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Carcinoma, Hepatocellular
Autophagy restricts mitochondrial DNA damage-induced release of ENDOG (endonuclease G) to regulate genome stability.
Carcinoma, Hepatocellular
Genomic DNA double-strand breaks are targets for hepadnaviral DNA integration.
Choriocarcinoma
HSPC117 is regulated by epigenetic modification and is involved in the migration of JEG-3 cells.
Coinfection
Phage T4 SegB protein is a homing endonuclease required for the preferred inheritance of T4 tRNA gene region occurring in co-infection with a related phage.
Decompression Sickness
Conformational changes and cleavage by the homing endonuclease I-PpoI: a critical role for a leucine residue in the active site.
Fetal Death
HSPC117 deficiency in cloned embryos causes placental abnormality and fetal death.
Hepatitis B
Autophagy restricts mitochondrial DNA damage-induced release of ENDOG (endonuclease G) to regulate genome stability.
Infections
Better late than early: delayed translation of intron-encoded endonuclease I-TevI is required for efficient splicing of its host group I intron.
Infertility
Comparison of Model Predictions and Laboratory Observations of Transgene Frequencies in Continuously-Breeding Mosquito Populations.
Leprosy
Characterization of Mycobacterium leprae RecA intein, a LAGLIDADG homing endonuclease, reveals a unique mode of DNA binding, helical distortion, and cleavage compared with a canonical LAGLIDADG homing endonuclease.
Malaria
A synthetic homing endonuclease-based gene drive system in the human malaria mosquito.
Malaria
A synthetic sex ratio distortion system for the control of the human malaria mosquito.
Malaria
Requirements for effective malaria control with homing endonuclease genes.
Microcephaly
Autosomal-Recessive Mutations in the tRNA Splicing Endonuclease Subunit TSEN15 Cause Pontocerebellar Hypoplasia and Progressive Microcephaly.
Nervous System Diseases
CLP1 as a novel player in linking tRNA splicing to neurodegenerative disorders.
Retinitis Pigmentosa
Toward genome editing in X-linked RP-development of a mouse model with specific treatment relevant features.
trna-intron lyase deficiency
HSPC117 deficiency in cloned embryos causes placental abnormality and fetal death.
Tuberculosis
Dynamics differentiate between active and inactive inteins.
Tuberculosis
Homing events in the gyrA gene of some mycobacteria.
Tuberculosis
In vitro splicing of erythropoietin by the Mycobacterium tuberculosis RecA intein without substituting amino acids at the splice junctions.
Tuberculosis
Mycobacterium tuberculosis RecA intein, a LAGLIDADG homing endonuclease, displays Mn(2+) and DNA-dependent ATPase activity.
Vaccinia
Classification, diagnosis and potential mechanisms in Pontocerebellar Hypoplasia.
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brenda
Q8NCE0: subunit Sen2, Q9BSV6: subunit Sen34, Q8WW01: subunit Sen15, Q7Z6J9: subunit 54
SwissProt
brenda
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brenda
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brenda
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physiological function
EndA function is required for brain development in humans. Human EndA complex (TSen2, TSen15, TSen34, and TSen54) cleaves introns from pre-tRNAs, and the TSen2 subunit is involved in pre-mRNA'3 end formation
malfunction
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epleting HSPC117 by RNA interference abolishes interstrand ligation and impaires processing of pre-tRNA transcripts into mature tRNA in cell extracts
malfunction
mutations in human EndA cause neurological disorders including pontocerebellar hypoplasia and progressive microcephaly
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SEN2_HUMAN
465
0
53247
Swiss-Prot
other Location (Reliability: 4 )
SEN34_HUMAN
310
0
33652
Swiss-Prot
other Location (Reliability: 3 )
A0A7P0T8Z6_HUMAN
317
0
35601
TrEMBL
other Location (Reliability: 4 )
E7EQB3_HUMAN
315
0
34318
TrEMBL
Mitochondrion (Reliability: 5 )
A0A7P0T9K6_HUMAN
390
0
44435
TrEMBL
other Location (Reliability: 4 )
A0A7P0Z4J6_HUMAN
434
0
49267
TrEMBL
other Location (Reliability: 4 )
B4DT51_HUMAN
312
0
33892
TrEMBL
other Location (Reliability: 3 )
H7C2Z3_HUMAN
490
0
55307
TrEMBL
other Location (Reliability: 4 )
A0A7P0T8I7_HUMAN
311
0
35306
TrEMBL
other Location (Reliability: 4 )
H7C301_HUMAN
322
0
36421
TrEMBL
other Location (Reliability: 4 )
A0A7P0TA86_HUMAN
423
0
48278
TrEMBL
other Location (Reliability: 4 )
C9J7Z4_HUMAN
516
0
58612
TrEMBL
other Location (Reliability: 4 )
A0A7P0TBE6_HUMAN
460
0
52571
TrEMBL
other Location (Reliability: 4 )
A0A024R2G3_HUMAN
465
0
53247
TrEMBL
other Location (Reliability: 4 )
A0A7P0T914_HUMAN
484
0
55500
TrEMBL
other Location (Reliability: 4 )
A0A7P0T841_HUMAN
473
0
53325
TrEMBL
other Location (Reliability: 4 )
A0A7P0Z492_HUMAN
422
0
47960
TrEMBL
other Location (Reliability: 4 )
A0A7P0T8F8_HUMAN
458
0
52195
TrEMBL
other Location (Reliability: 4 )
B7Z355_HUMAN
438
0
50225
TrEMBL
other Location (Reliability: 4 )
A0A7P0T9Y6_HUMAN
373
0
42444
TrEMBL
other Location (Reliability: 4 )
A0A590UJW4_HUMAN
312
0
33874
TrEMBL
other Location (Reliability: 3 )
A0A0G2JNL0_HUMAN
251
0
27280
TrEMBL
Mitochondrion (Reliability: 5 )
A0A024R4N9_HUMAN
310
0
33652
TrEMBL
other Location (Reliability: 3 )
A0A7P0T8S9_HUMAN
312
0
35368
TrEMBL
other Location (Reliability: 4 )
A8K568_HUMAN
465
0
53277
TrEMBL
other Location (Reliability: 4 )
A0A7P0T923_HUMAN
347
0
39140
TrEMBL
other Location (Reliability: 4 )
A0A590UJ73_HUMAN
303
0
33037
TrEMBL
other Location (Reliability: 3 )
A0A0G2JNK5_HUMAN
252
0
27337
TrEMBL
Mitochondrion (Reliability: 5 )
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oligomer
human EndA complex: 53247 (subunit TSen2) + 18641 (subunit TSen15) + 33652 (subunit TSen34) + 58819 (subunit TSen54), calculated from sequence
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chromatographic methods
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expression in Eschericha coli
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Stange, N.; Gross, H.J.; Beier, H.
Wheat germ splicing endonuclease is highly specific for plant pre-tRNAs
EMBO J.
7
3823-3828
1988
Homo sapiens, Triticum aestivum
brenda
Budde, B.S.; Namavar, Y.; Barth, P.G.; Poll-The, B.T.; Nuernberg, G.; Becker, C.; van Ruissen, F.; Weterman, M.A.; Fluiter, K.; te Beek, E.T.; et al.
tRNA splicing endonuclease mutations cause pontocerebellar hypoplasia
Nat. Genet.
40
1113-1118
2008
Homo sapiens
brenda
Weitzer, S.; Martinez, J.
hClp1: a novel kinase revitalizes RNA metabolism
Cell Cycle
6
2133-2137
2007
Homo sapiens
brenda
Weitzer, S.; Martinez, J.
The human RNA kinase hClp1 is active on 3 transfer RNA exons and short interfering RNAs
Nature
447
222-226
2007
Homo sapiens
brenda
Popow, J.; Englert, M.; Weitzer, S.; Schleiffer, A.; Mierzwa, B.; Mechtler, K.; Trowitzsch, S.; Will, C.L.; Luehrmann, R.; Soell, D.; Martinez, J.
HSPC117 is the essential subunit of a human tRNA splicing ligase complex
Science
331
760-764
2011
Homo sapiens
brenda
Hirata, A.
Recent insights into the structure, function, and evolution of the RNA-splicing endonucleases
Front. Genet.
10
103
2019
Candidatus Micrarchaeum acidiphilum ARMAN-2 (C7DIA5), Archaeoglobus fulgidus (O29362), Saccharomyces cerevisiae (P16658 AND P39707 AND Q04675 AND Q02825), Saccharomyces cerevisiae, Methanocaldococcus jannaschii (Q58819), Nanoarchaeum equitans (Q74MP4), Homo sapiens (Q8NCE0 AND Q9BSV6 AND Q8WW01 AND Q7Z6J9), Homo sapiens, Methanopyrus kandleri (Q8TGZ5), Pyrobaculum aerophilum (Q8ZVI1), Thermoplasma acidophilum (Q9HIY5), Aeropyrum pernix (Q9YE85), Aeropyrum pernix ATCC 700893 (Q9YE85), Archaeoglobus fulgidus ATCC 49558 (O29362), Methanocaldococcus jannaschii ATCC 43067 (Q58819), Saccharomyces cerevisiae ATCC 204508 (P16658 AND P39707 AND Q04675 AND Q02825), Pyrobaculum aerophilum ATCC 51768 (Q8ZVI1), Thermoplasma acidophilum ATCC 25905 (Q9HIY5)
brenda