Reactome: A Curated Pathway Database
THIS SITE IS USED FOR CURATION AND TESTING
IT IS NOT STABLE, IS LINKED TO AN INCOMPLETE DATA SET, AND IS NOT MONITORED FOR PERFORMANCE. WE STRONGLY RECOMMEND THE USE OF OUR PUBLIC SITE

Query author contributions in Reactome

Reactome depends on collaboration between our curation team and outside experts to assemble and peer-review its pathway modules. The integration of ORCID within Reactome enables us to meet a key challenge with authoring, curating and reviewing biological information by incentivizing and crediting the external experts that contribute their expertise and time to the Reactome curation process. More information is available at ORCID and Reactome.

If you have an ORCID ID that is not listed on this page, please forward this information to us and we will update your Reactome pathway records.

Name Email address

Details on Person UniProt:Q9BQ52 ELAC2

Class:IdReferenceGeneProduct:153122
_chainChangeLogtransit peptide:1-16 added on Sat February 7 2015;chain:17-826 added on Sat February 7 2015
_displayNameUniProt:Q9BQ52 ELAC2
_timestamp2025-02-21 19:42:34
chaintransit peptide:1-16
chain:17-826
checksum4AE701C755EC7339
commentFUNCTION Zinc phosphodiesterase, which displays mitochondrial tRNA 3'-processing endonuclease activity. Involved in tRNA maturation, by removing a 3'-trailer from precursor tRNA (PubMed:21593607). Associates with mitochondrial DNA complexes at the nucleoids to initiate RNA processing and ribosome assembly (PubMed:24703694).CATALYTIC ACTIVITY Endonucleolytic cleavage of RNA, removing extra 3' nucleotides from tRNA precursor, generating 3' termini of tRNAs. A 3'-hydroxy group is left at the tRNA terminus and a 5'-phosphoryl group is left at the trailer molecule.COFACTOR Homodimer (By similarity). Interacts with PTCD1.SUBCELLULAR LOCATION Mainly mitochondrial.ALTERNATIVE PRODUCTS Widely expressed. Highly expressed in heart, placenta, liver, skeletal muscle, kidney, pancreas, testis and ovary. Weakly expressed in brain, lung, spleen, thymus, prostate, small intestine, colon and leukocytes.DISEASE The disease is caused by variants affecting the gene represented in this entry.DISEASE The disease is caused by variants affecting the gene represented in this entry.SIMILARITY Belongs to the RNase Z family.
created[InstanceEdit:143527] Schmidt, EE, 2004-11-12 07:45:10
descriptionrecommendedName: Zinc phosphodiesterase ELAC protein 2 ecNumber: 3.1.26.11 alternativeName: ElaC homolog protein 2 alternativeName: Heredity prostate cancer protein 2 alternativeName: Ribonuclease Z 2 shortName: RNase Z 2 alternativeName: tRNA 3 endonuclease 2 alternativeName: tRNase Z 2
geneNameELAC2
HPC2
identifierQ9BQ52
isSequenceChangedFALSE
keyword3D-structure
Alternative splicing
Disease variant
Endonuclease
Hydrolase
Metal-binding
Mitochondrion
Mitochondrion nucleoid
Nuclease
Nucleus
Phosphoprotein
Primary mitochondrial disease
Proteomics identification
Proto-oncogene
Reference proteome
Transit peptide
tRNA processing
Zinc
modified[InstanceEdit:9836292] Weiser, Joel, 2023-05-25
[InstanceEdit:9852000] Weiser, Joel, 2023-11-03
[InstanceEdit:9926675] Weiser, Joel, 2024-11-03
[InstanceEdit:9939033] Weiser, Joel, 2025-02-21
nameELAC2
referenceDatabase[ReferenceDatabase:2] UniProt
referenceGene[ReferenceDNASequence:8962107] ENSEMBL:ENSG00000006744 ELAC2 [Homo sapiens]
secondaryIdentifierRNZ2_HUMAN
B4DPL9
Q6IA94
Q9HAS8
Q9HAS9
Q9NVT1
sequenceLength826
species[Species:48887] Homo sapiens
(isoformParent)[ReferenceIsoform:153123] UniProt:Q9BQ52-2 ELAC2 [Homo sapiens]
[ReferenceIsoform:153124] UniProt:Q9BQ52-3 ELAC2 [Homo sapiens]
[ReferenceIsoform:413329] UniProt:Q9BQ52-1 ELAC2 [Homo sapiens]
[ReferenceIsoform:8974876] UniProt:Q9BQ52-4 ELAC2 [Homo sapiens]
(referenceEntity)[EntityWithAccessionedSequence:5696766] ELAC2 [nucleoplasm] [Homo sapiens]
[EntityWithAccessionedSequence:6786837] ELAC2 [mitochondrial matrix] [Homo sapiens]
[EntityWithAccessionedSequence:9708824] ELAC2 [cytosol] [Homo sapiens]
[Change default viewing format]
No pathways have been reviewed or authored by UniProt:Q9BQ52 ELAC2 (153122)