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Abetalipoproteinemia
Chorea, psychosis, acanthocytosis, and prolonged survival associated with ELAC2 mutations.
Abscess
Genes Contributing to Porphyromonas gingivalis Fitness in Abscess and Epithelial Cell Colonization Environments.
Acidosis, Lactic
Mutations in ELAC2 associated with hypertrophic cardiomyopathy impair mitochondrial tRNA 3'-end processing.
Carcinogenesis
Mutations in ELAC2 associated with hypertrophic cardiomyopathy impair mitochondrial tRNA 3'-end processing.
Carcinogenesis
The product of the candidate prostate cancer susceptibility gene ELAC2 interacts with the gamma-tubulin complex.
Carcinoma
Association of hereditary prostate cancer gene polymorphic variants with sporadic aggressive prostate carcinoma.
Cardiomyopathies
ELAC2/RNaseZ-linked cardiac hypertrophy in Drosophila melanogaster.
Cardiomyopathies
The Phenotype and Outcome of Infantile Cardiomyopathy Caused by a Homozygous ELAC2 Mutation.
Cardiomyopathy, Dilated
Concerted regulation of mitochondrial and nuclear non-coding RNAs by a dual-targeted RNase Z.
Cardiomyopathy, Hypertrophic
A homozygous splicing mutation in ELAC2 suggests phenotypic variability including intellectual disability with minimal cardiac involvement.
Cardiomyopathy, Hypertrophic
ELAC2 Mutations Cause a Mitochondrial RNA Processing Defect Associated with Hypertrophic Cardiomyopathy.
Cardiomyopathy, Hypertrophic
Mutations in ELAC2 associated with hypertrophic cardiomyopathy impair mitochondrial tRNA 3'-end processing.
Chorea
Chorea, psychosis, acanthocytosis, and prolonged survival associated with ELAC2 mutations.
Infections
ELAC2, an Enzyme for tRNA Maturation, Plays a Role in the Cleavage of a Mature tRNA to Produce a tRNA-Derived RNA Fragment During Respiratory Syncytial Virus Infection.
Infections
Genes Contributing to Porphyromonas gingivalis Fitness in Abscess and Epithelial Cell Colonization Environments.
Infertility, Male
The structural characteristics and the substrate recognition properties of RNase ZS1.
Infertility, Male
The transcription factor GATA10 regulates fertility conversion of a two-line hybrid tms5 mutant rice via the modulation of UbL40 expression.
Intellectual Disability
A homozygous splicing mutation in ELAC2 suggests phenotypic variability including intellectual disability with minimal cardiac involvement.
Mitochondrial Diseases
Mutations in ELAC2 associated with hypertrophic cardiomyopathy impair mitochondrial tRNA 3'-end processing.
Mitochondrial Diseases
Naturally Occurring Mutations in Human Mitochondrial Pre-tRNASer(UCN) Can Affect the Transfer Ribonuclease Z Cleavage Site, Processing Kinetics, and Substrate Secondary Structure.
nadh:ubiquinone reductase (h+-translocating) deficiency
A homozygous splicing mutation in ELAC2 suggests phenotypic variability including intellectual disability with minimal cardiac involvement.
nadh:ubiquinone reductase (h+-translocating) deficiency
ELAC2 Mutations Cause a Mitochondrial RNA Processing Defect Associated with Hypertrophic Cardiomyopathy.
Neoplasm Metastasis
Nuclear ELAC2 overexpression is associated with increased hazard for relapse after radical prostatectomy.
Neoplasms
A novel class of small RNAs: tRNA-derived RNA fragments (tRFs).
Neoplasms
ELAC2 polymorphisms and prostate cancer risk: a meta-analysis based on 18 case-control studies.
Neoplasms
Genetic determinants of prostate cancer: a review.
Neoplasms
Nuclear ELAC2 overexpression is associated with increased hazard for relapse after radical prostatectomy.
Neoplasms
Prostate cancer: simplicity to complexity.
Neoplasms
Single and multivariate associations of MSR1, ELAC2, and RNASEL with prostate cancer in an ethnic diverse cohort of men.
Neoplasms
The product of the candidate prostate cancer susceptibility gene ELAC2 interacts with the gamma-tubulin complex.
Neoplasms
TRUE Gene Silencing: Screening of a Heptamer-type Small Guide RNA Library for Potential Cancer Therapeutic Agents.
Pericardial Effusion
The Phenotype and Outcome of Infantile Cardiomyopathy Caused by a Homozygous ELAC2 Mutation.
Prostatic Diseases
ELAC2 polymorphisms and prostate cancer risk: a meta-analysis based on 18 case-control studies.
Prostatic Neoplasms
A candidate prostate cancer susceptibility gene at chromosome 17p.
Prostatic Neoplasms
A candidate prostate cancer susceptibility gene encodes tRNA 3' processing endoribonuclease.
Prostatic Neoplasms
A novel class of small RNAs: tRNA-derived RNA fragments (tRFs).
Prostatic Neoplasms
A Study of Ser217Leu and Ala541Thr Polymorphism in the Men Afflicted with Prostate Cancer and in the Men being Suspicious of Prostate Cancer.
Prostatic Neoplasms
A survey of green plant tRNA 3'-end processing enzyme tRNase Zs, homologs of the candidate prostate cancer susceptibility protein ELAC2.
Prostatic Neoplasms
Association between RNASEL, MSR1, and ELAC2 single nucleotide polymorphisms and gene expression in prostate cancer risk.
Prostatic Neoplasms
Association of common missense changes in ELAC2 ( HPC2) with prostate cancer in a Japanese case-control series.
Prostatic Neoplasms
Association of hereditary prostate cancer gene polymorphic variants with sporadic aggressive prostate carcinoma.
Prostatic Neoplasms
Characterization of linkage disequilibrium structure, mutation history, and tagging SNPs, and their use in association analyses: ELAC2 and familial early-onset prostate cancer.
Prostatic Neoplasms
Characterization of TRZ1, a yeast homolog of the human candidate prostate cancer susceptibility gene ELAC2 encoding tRNase Z.
Prostatic Neoplasms
ELAC2 and prostate cancer risk in Afro-Caribbeans of Tobago.
Prostatic Neoplasms
ELAC2 polymorphisms and prostate cancer risk: a meta-analysis based on 18 case-control studies.
Prostatic Neoplasms
ELAC2, a putative prostate cancer susceptibility gene product, potentiates TGF-beta/Smad-induced growth arrest of prostate cells.
Prostatic Neoplasms
ELAC2/HPC2 polymorphisms, prostate-specific antigen levels, and prostate cancer.
Prostatic Neoplasms
Genetic analysis of the principal genes related to prostate cancer: A review.
Prostatic Neoplasms
Genetic determinants of prostate cancer: a review.
Prostatic Neoplasms
Genome-wide scan for prostate cancer susceptibility genes using families from the University of Michigan prostate cancer genetics project finds evidence for linkage on chromosome 17 near BRCA1.
Prostatic Neoplasms
Identification and analysis of candidate fungal tRNA 3'-end processing endonucleases tRNase Zs, homologs of the putative prostate cancer susceptibility protein ELAC2.
Prostatic Neoplasms
Integration of copy number and transcriptomics provides risk stratification in prostate cancer: A discovery and validation cohort study.
Prostatic Neoplasms
Meta-analysis of associations of the Ser217Leu and Ala541Thr variants in ELAC2 (HPC2) and prostate cancer.
Prostatic Neoplasms
Molecular biology in prostate cancer.
Prostatic Neoplasms
Mutation screening and association study of RNASEL as a prostate cancer susceptibility gene.
Prostatic Neoplasms
Mutational analysis of susceptibility genes RNASEL/HPC1, ELAC2/HPC2, and MSR1 in sporadic prostate cancer.
Prostatic Neoplasms
Mutations in ELAC2 associated with hypertrophic cardiomyopathy impair mitochondrial tRNA 3'-end processing.
Prostatic Neoplasms
Nuclear ELAC2 overexpression is associated with increased hazard for relapse after radical prostatectomy.
Prostatic Neoplasms
Nucleases of the metallo-beta-lactamase family and their role in DNA and RNA metabolism.
Prostatic Neoplasms
Perspective: prostate cancer susceptibility genes.
Prostatic Neoplasms
Prevalence of the Ser217Leu Variant of the ELAC2 Gene and Its Association with Prostate Cancer in Population of the Littoral Region of Cameroon.
Prostatic Neoplasms
Prevalent mutations in prostate cancer.
Prostatic Neoplasms
Prognostic role of genetic biomarkers in clinical progression of prostate cancer.
Prostatic Neoplasms
Sequence variants of elaC homolog 2 (Escherichia coli) (ELAC2) gene and susceptibility to prostate cancer in the Health Professionals Follow-Up Study.
Prostatic Neoplasms
Single and multivariate associations of MSR1, ELAC2, and RNASEL with prostate cancer in an ethnic diverse cohort of men.
Prostatic Neoplasms
Structure of primate and rodent orthologs of the prostate cancer susceptibility gene ELAC2.
Prostatic Neoplasms
The Caenorhabditis elegans homolog of the putative prostate cancer susceptibility gene ELAC2, hoe-1, plays a role in germline proliferation.
Prostatic Neoplasms
The missense mutations in the candidate prostate cancer gene ELAC2 do not alter enzymatic properties of its product.
Prostatic Neoplasms
The product of the candidate prostate cancer susceptibility gene ELAC2 interacts with the gamma-tubulin complex.
Respiratory Syncytial Virus Infections
ELAC2, an Enzyme for tRNA Maturation, Plays a Role in the Cleavage of a Mature tRNA to Produce a tRNA-Derived RNA Fragment During Respiratory Syncytial Virus Infection.
Severe Combined Immunodeficiency
Nucleases of the metallo-beta-lactamase family and their role in DNA and RNA metabolism.
Starvation
Retrograde nuclear import of tRNA precursors is required for modified base biogenesis in yeast.
Virus Diseases
ELAC2, an Enzyme for tRNA Maturation, Plays a Role in the Cleavage of a Mature tRNA to Produce a tRNA-Derived RNA Fragment During Respiratory Syncytial Virus Infection.
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0.000016
pre-tRNA(Arg)
-
-
-
0.0000043
pre-tRNA(Val)
-
-
-
0.000022 - 0.000132
pre-tRNAArg
-
0.000029
pre-tRNAGly
-
25 mM K-MOPS, 2 mM MgCl2, at pH 6.8 and 37°C
-
0.000018
pre-tRNAHis
-
25 mM K-MOPS, 2 mM MgCl2, at pH 6.8 and 37°C
-
0.000048
pre-tRNAIle
-
25 mM K-MOPS, 2 mM MgCl2, at pH 6.8 and 37°C
-
0.000024
pre-tRNALeu(UUR)
-
25 mM K-MOPS, 3 mM CaCl2, at pH 6.8 and 37°C
-
0.0000048
pre-tRNASer(UCN)
-
25 mM K-MOPS, pH 6.75, 2.5 mM CaCl2, 1 mM dithiothreitol, 5% glycerol, 4 U/ml RNasin, 100 microg/ml bovine serum albumin
-
0.0000633
pre-tRNASer(UCN)7443C
-
25 mM K-MOPS, pH 6.75, 2.5 mM CaCl2, 1 mM dithiothreitol, 5% glycerol, 4 U/ml RNasin, 100 microg/ml bovine serum albumin
-
0.0000069
pre-tRNASer(UCN)7444U
-
25 mM K-MOPS, pH 6.75, 2.5 mM CaCl2, 1 mM dithiothreitol, 5% glycerol, 4 U/ml RNasin, 100 microg/ml bovine serum albumin
-
0.0000197
pre-tRNASer(UCN)7445C
-
25 mM K-MOPS, pH 6.75, 2.5 mM CaCl2, 1 mM dithiothreitol, 5% glycerol, 4 U/ml RNasin, 100 microg/ml bovine serum albumin
-
0.0000855
pre-tRNASer(UCN)7445G
-
25 mM K-MOPS, pH 6.75, 2.5 mM CaCl2, 1 mM dithiothreitol, 5% glycerol, 4 U/ml RNasin, 100 microg/ml bovine serum albumin
-
0.0000108
pre-tRNASer(UCN)7510G
-
25 mM K-MOPS, pH 6.75, 2.5 mM CaCl2, 1 mM dithiothreitol, 5% glycerol, 4 U/ml RNasin, 100 microg/ml bovine serum albumin
-
0.0000076
pre-tRNASer(UCN)7511G
-
25 mM K-MOPS, pH 6.75, 2.5 mM CaCl2, 1 mM dithiothreitol, 5% glycerol, 4 U/ml RNasin, 100 microg/ml bovine serum albumin
-
0.000004
pre-tRNASer(UCN)7512G
-
25 mM K-MOPS, pH 6.75, 2.5 mM CaCl2, 1 mM dithiothreitol, 5% glycerol, 4 U/ml RNasin, 100 microg/ml bovine serum albumin
-
0.000022
pre-tRNAArg
-
25 mM K-MOPS, pH 6.75, 2 mM MgCl2, 1 mM dithiothreitol, 5% glycerol, 4 U/ml RNasin, 100 microg/ml bovine serum albumin
-
0.000132
pre-tRNAArg
-
25 mM K-MOPS, pH 6.75, 1 mM MnCl2, 1 mM dithiothreitol, 5% glycerol, 4 U/ml RNasin, 100 microg/ml bovine serum albumin
-
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0.021 - 6.18
pre-tRNAArg
-
0.035
pre-tRNAGly
-
25 mM K-MOPS, 2 mM MgCl2, at pH 6.8 and 37°C
-
0.237
pre-tRNAHis
-
25 mM K-MOPS, 2 mM MgCl2, at pH 6.8 and 37°C
-
0.018
pre-tRNAIle
-
25 mM K-MOPS, 2 mM MgCl2, at pH 6.8 and 37°C
-
0.25
pre-tRNALeu(UUR)
-
25 mM K-MOPS, 3 mM CaCl2, at pH 6.8 and 37°C
-
0.07
pre-tRNASer(UCN)
-
25 mM K-MOPS, pH 6.75, 2.5 mM CaCl2, 1 mM dithiothreitol, 5% glycerol, 4 U/ml RNasin, 100 microg/ml bovine serum albumin
-
0.23
pre-tRNASer(UCN)7443C
-
25 mM K-MOPS, pH 6.75, 2.5 mM CaCl2, 1 mM dithiothreitol, 5% glycerol, 4 U/ml RNasin, 100 microg/ml bovine serum albumin
-
0.153
pre-tRNASer(UCN)7444U
-
25 mM K-MOPS, pH 6.75, 2.5 mM CaCl2, 1 mM dithiothreitol, 5% glycerol, 4 U/ml RNasin, 100 microg/ml bovine serum albumin
-
0.012
pre-tRNASer(UCN)7445C
-
25 mM K-MOPS, pH 6.75, 2.5 mM CaCl2, 1 mM dithiothreitol, 5% glycerol, 4 U/ml RNasin, 100 microg/ml bovine serum albumin
-
0.345
pre-tRNASer(UCN)7445G
-
25 mM K-MOPS, pH 6.75, 2.5 mM CaCl2, 1 mM dithiothreitol, 5% glycerol, 4 U/ml RNasin, 100 microg/ml bovine serum albumin
-
0.05
pre-tRNASer(UCN)7510G, pre-tRNASer(UCN)7511G, pre-tRNASer(UCN)7512G
-
25 mM K-MOPS, pH 6.75, 2.5 mM CaCl2, 1 mM dithiothreitol, 5% glycerol, 4 U/ml RNasin, 100 microg/ml bovine serum albumin
-
0.021
pre-tRNAArg
-
25 mM K-MOPS, pH 6.75, 1 mM MnCl2, 1 mM dithiothreitol, 5% glycerol, 4 U/ml RNasin, 100 microg/ml bovine serum albumin
-
6.18
pre-tRNAArg
-
25 mM K-MOPS, pH 6.75, 2 mM MgCl2, 1 mM dithiothreitol, 5% glycerol, 4 U/ml RNasin, 100 microg/ml bovine serum albumin
-
Please wait a moment until the data is sorted. This message will disappear when the data is sorted.
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Please wait a moment until the data is sorted. This message will disappear when the data is sorted.
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1641insG
-
frameshift mutation of ELAC2, lacks the C-terminal half, cannot increase suppression of ade6-704 in strain yYH1
D515A
-
tested for activity in presence of Mg2+ or Mn2+
D550A
-
Asp(I), active site residue
D666A
-
Asp(II), active site residue
E518A
-
tested for activity in presence of Mg2+ or Mn2+
E89A
-
influence on activity tested
F109S
-
influence on activity tested
G120Y
-
influence on activity tested
G281Y
-
influence on activity tested
G554L
-
tested for activity in presence of Mg2+ or Mn2+
H548A
-
His(II), active site residue
H551A
-
His(III), active site residue
H685A
-
tested for activity in presence of Mg2+ or Mn2+
H702A
-
His(V), active site residue
H724A
-
His(VI), active site residue
K282A
-
influence on activity tested
L539A
-
tested for activity in presence of Mg2+ or Mn2+
P493A
-
tested for activity in presence of Mg2+ or Mn2+
R497A
-
tested for activity in presence of Mg2+ or Mn2+
R718H
-
seems to be associated with the occurrence of prostate cancer
R781H
-
overexpression of this missense mutant of ELAC2 can increase suppression of ade6-704 in strain yYH1
T520Q
-
tested for activity in presence of Mg2+ or Mn2+
A541T
-
seems to be associated with the occurrence of prostate cancer
A541T
-
overexpression of this missense mutant of ELAC2 can increase suppression of ade6-704 in strain yYH1
H546A
-
His(I), active site residue
H546A
-
ELAC2 mutation, cannot increase suppression of ade6-704 in strain yYH1
S217L
-
seems to be associated with the occurrence of prostate cancer
S217L
-
overexpression of this missense mutant of ELAC2 can increase suppression of ade6-704 in strain yYH1
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Nashimoto, M.
Distribution of both lengths and 5' terminal nucleotides of mammalian pre-tRNA 3' trailers reflects properties of 3' processing endoribonuclease
Nucleic Acids Res.
25
1148-1154
1997
Homo sapiens, Mus musculus, Sus scrofa
brenda
Mrl, M.; Marchfelder, A.
The final cut. The importance of tRNA 3'-processing
EMBO Rep.
2
17-20
2001
Saccharomyces cerevisiae, Homo sapiens, Physarum sp., Rattus norvegicus
brenda
Shibata, H.S.; Minagawa, A.; Takaku, H.; Takagi, M.; Nashimoto, M.
Unstructured RNA is a substrate for tRNase Z
Biochemistry
45
5486-5492
2006
Bacillus subtilis, Escherichia coli, Homo sapiens, Pyrobaculum aerophilum, Saccharomyces cerevisiae, Sus scrofa, Thermotoga maritima
brenda
Vogel, A.; Schilling, O.; Spath, B.; Marchfelder, A.
The tRNase Z family of proteins: physiological functions, substrate specificity and structural properties
Biol. Chem.
386
1253-1264
2005
Arabidopsis thaliana, Arabidopsis thaliana (Q8L633), Arabidopsis thaliana (Q8LGU7), Arabidopsis thaliana (Q8VYS2), Bacillus subtilis (P54548), Caenorhabditis elegans (O44476), Drosophila melanogaster (Q8MKW7), Escherichia coli (P0A8V0), Haloferax volcanii, Homo sapiens (Q9BQ52), Homo sapiens (Q9H777), Methanocaldococcus jannaschii (Q58897), Pyrobaculum aerophilum (Q8ZTJ7), Pyrococcus furiosus (Q8U182), Saccharomyces cerevisiae (P36159), Thermoplasma acidophilum (Q9HJ19), Thermotoga maritima
brenda
Yan, H.; Zareen, N.; Levinger, L.
Naturally occurring mutations in human mitochondrial pre-tRNASer(UCN) can affect the transfer ribonuclease Z cleavage site, processing kinetics, and substrate secondary structure
J. Biol. Chem.
281
3926-3935
2006
Homo sapiens
brenda
Spaeth, B.; Canino, G.; Marchfelder, A.
tRNase Z: the end is not in sight
Cell. Mol. Life Sci.
64
2404-2412
2007
Arabidopsis thaliana, Caenorhabditis elegans, Danio rerio, Drosophila melanogaster (Q8MKW7), Gallus gallus, Homo sapiens, Mus musculus, Neurospora crassa, Saccharomyces cerevisiae, Schizosaccharomyces pombe
brenda
Dominski, Z.
Nucleases of the metallo-beta-lactamase family and their role in DNA and RNA metabolism
Crit. Rev. Biochem. Mol. Biol.
42
67-93
2007
Escherichia coli, Homo sapiens
brenda
Ceballos, M.; Vioque, A.
tRNase Z
Protein Pept. Lett.
14
137-145
2007
Synechocystis sp., Arabidopsis thaliana, Bacillus subtilis, Escherichia coli, Homo sapiens, Methanocaldococcus jannaschii, Thermotoga maritima
brenda
Elbarbary, R.A.; Takaku, H.; Nashimoto, M.
Functional analyses for tRNase Z variants: an aspartate and a histidine in the active site are essential for the catalytic activity
Biochim. Biophys. Acta
1784
2079-2085
2008
Bacillus subtilis, Escherichia coli, Homo sapiens, Mus musculus
brenda
Takahashi, M.; Takaku, H.; Nashimoto, M.
Regulation of the human tRNase ZS gene expression
FEBS Lett.
582
2532-2536
2008
Homo sapiens
brenda
Hopkinson, A.; Levinger, L.
Effects of conserved D/T loop substitutions in the pre-tRNA substrate on tRNase Z catalysis
RNA Biol.
5
104-111
2008
Homo sapiens
brenda
Zhao, Z.; Su, W.; Yuan, S.; Huang, Y.
Functional conservation of tRNase ZL among Saccharomyces cerevisiae, Schizosaccharomyces pombe and humans
Biochem. J.
422
483-492
2009
Homo sapiens, Saccharomyces cerevisiae, Schizosaccharomyces pombe (P87168), Schizosaccharomyces pombe (Q10155), Schizosaccharomyces pombe
brenda
Elbarbary, R.A.; Takaku, H.; Uchiumi, N.; Tamiya, H.; Abe, M.; Nishida, H.; Nashimoto, M.
Human cytosolic tRNase ZL can downregulate gene expression through miRNA
FEBS Lett.
583
3241-3246
2009
Homo sapiens
brenda
Elbarbary, R.A.; Takaku, H.; Uchiumi, N.; Tamiya, H.; Abe, M.; Takahashi, M.; Nishida, H.; Nashimoto, M.
Modulation of gene expression by human cytosolic tRNase Z(L) through 5-half-tRNA
PLoS ONE
4
e5908
2009
Homo sapiens
brenda
Rossmanith, W.
Localization of human RNase Z isoforms: dual nuclear/mitochondrial targeting of the ELAC2 gene product by alternative translation initiation
PLoS ONE
6
e19152
2011
Homo sapiens
brenda
Brzezniak, L.; Bijata, M.; Szczesny, R.; Stepien, P.
Involvement of human ELAC2 gene product in 3' end processing of mitochondrial tRNAs
RNA Biol.
8
616-626
2011
Homo sapiens
brenda
Wang, Z.; Zheng, J.; Zhang, X.; Peng, J.; Liu, J.; Huang, Y.
Identification and sequence analysis of metazoan tRNA 3-end processing enzymes tRNase ZS
PLoS ONE
7
e44264
2012
Amphimedon queenslandica, Bombyx mori, Branchiostoma floridae, Caenorhabditis elegans, Canis lupus familiaris, Capitella teleta, Ciona intestinalis, Danio rerio, Drosophila melanogaster, Homo sapiens, Hydra vulgaris, Lottia gigantea, Loxodonta africana, Monosiga brevicollis, Mus musculus, Nematostella vectensis, Salmo salar, Strongylocentrotus purpuratus, Tribolium castaneum, Trichoplax adhaerens, Xenopus tropicalis
brenda
Wilson, C.; Ramai, D.; Serjanov, D.; Lama, N.; Levinger, L.; Chang, E.J.
Tethered domains and flexible regions in tRNase Z(L), the long form of tRNase Z
PLoS ONE
8
e66942
2013
Drosophila melanogaster, Homo sapiens
brenda
Levinger, L.; Serjanov, D.
Pathogenesis-related mutations in the T-loops of human mitochondrial tRNAs affect 3' end processing and tRNA structure
RNA Biol.
9
283-291
2012
Homo sapiens
brenda
Ninomiya, S.; Kawano, M.; Abe, T.; Ishikawa, T.; Takahashi, M.; Tamura, M.; Takahashi, Y.; Nashimoto, M.
Potential small guide RNAs for tRNase ZL from human plasma, peripheral blood mononuclear cells, and cultured cell lines
PLoS ONE
10
e0118631
2015
Homo sapiens
brenda