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Details on Person Mitochondrial RNase P (mtRNase P) cleaves the 5' ends of pre-tRNAs and ELAC2 (RNase Z) cleaves the 3' ends of pre-tRNAs in the H strand transcript
Class:Id BlackBoxEvent:6785722
_displayName Mitochondrial RNase P (mtRNase P) cleaves the 5' ends of pre-tRNAs and ELAC2 (RNase Z) cleaves the 3' ends of pre-tRNAs in the H strand transcript
_doRelease TRUE
_timestamp 2026-03-31 19:44:43
authored [InstanceEdit:6785497] May, Bruce, 2015-06-29
catalystActivity [CatalystActivity:6786864] tRNA-specific ribonuclease activity of ELAC2 [mitochondrial matrix]
catalystActivityReference [CatalystActivityReference:9643122] tRNA-specific ribonuclease activity of ELAC2 [mitochondrial matrix] Involvement of human ELAC2 gene product in 3' end processing of mitochondrial tRNAs
compartment [Compartment:5460] mitochondrial matrix
created [InstanceEdit:6785712] May, Bruce, 2015-06-30
edited [InstanceEdit:6785497] May, Bruce, 2015-06-29
goBiologicalProcess [GO_BiologicalProcess:6788631] mitochondrial tRNA processing
input [GenomeEncodedEntity:6786802] H strand transcript [mitochondrial matrix] [Homo sapiens]
isChimeric FALSE
literatureReference [LiteratureReference:6785710] RNase P without RNA: identification and functional reconstitution of the human mitochondrial tRNA processing enzyme [LiteratureReference:6785719] Structure of the nuclease subunit of human mitochondrial RNase P [LiteratureReference:6786883] Auto-inhibitory Mechanism of the Human Mitochondrial RNase P Protein Complex [LiteratureReference:6786810] Molecular insights into HSD10 disease: impact of SDR5C1 mutations on the human mitochondrial RNase P complex [LiteratureReference:6786889] A subcomplex of human mitochondrial RNase P is a bifunctional methyltransferase--extensive moonlighting in mitochondrial tRNA biogenesis [LiteratureReference:6786834] Mitochondrial ribonuclease P structure provides insight into the evolution of catalytic strategies for precursor-tRNA 5' processing [LiteratureReference:6784555] Involvement of human ELAC2 gene product in 3' end processing of mitochondrial tRNAs [LiteratureReference:5696733] Localization of human RNase Z isoforms: dual nuclear/mitochondrial targeting of the ELAC2 gene product by alternative translation initiation [LiteratureReference:6786797] ELAC2 mutations cause a mitochondrial RNA processing defect associated with hypertrophic cardiomyopathy [LiteratureReference:6785708] RNA processing in human mitochondria [LiteratureReference:6792957] Mitochondrial transcript maturation and its disorders [LiteratureReference:6797518] Pathogenesis-related mutations in the T-loops of human mitochondrial tRNAs affect 3' end processing and tRNA structure
modified [InstanceEdit:6786890] May, Bruce, 2015-07-10 [InstanceEdit:6787743] May, Bruce, 2015-07-16 [InstanceEdit:6788634] May, Bruce, 2015-07-23 [InstanceEdit:6788719] May, Bruce, 2015-07-24 [InstanceEdit:6793023] May, Bruce, 2015-08-28 [InstanceEdit:6797521] May, Bruce, 2015-09-13 [InstanceEdit:6803013] May, Bruce, 2015-10-04 [InstanceEdit:6805199] May, Bruce, 2015-10-19 [InstanceEdit:6806694] May, Bruce, 2015-10-24 [InstanceEdit:6808657] May, Bruce, 2015-11-07 [InstanceEdit:6810572] May, Bruce, 2015-11-18 [InstanceEdit:8856102] May, Bruce, 2016-02-09 [InstanceEdit:8864145] May, Bruce, 2016-03-12 [InstanceEdit:9643047] Wu, G, 2019-04-04 [InstanceEdit:9685233] May, Bruce, 2020-04-23 [InstanceEdit:9830342] Matthews, Lisa, 2023-03-08 [InstanceEdit:9986398] May, Bruce, 2026-03-31
name Mitochondrial RNase P (mtRNase P) cleaves the 5' ends of pre-tRNAs and ELAC2 (RNase Z) cleaves the 3' ends of pre-tRNAs in the H strand transcript
output [EntityWithAccessionedSequence:6792974] 12S rRNA [mitochondrial matrix] [Homo sapiens] [EntityWithAccessionedSequence:6792998] 16S rRNA [mitochondrial matrix] [Homo sapiens] [EntityWithAccessionedSequence:6792964] MT-ATP6 mRNA [mitochondrial matrix] [Homo sapiens] [EntityWithAccessionedSequence:6793010] MT-ATP8 mRNA [mitochondrial matrix] [Homo sapiens] [EntityWithAccessionedSequence:6793012] MT-CO1 mRNA [mitochondrial matrix] [Homo sapiens] [EntityWithAccessionedSequence:6792984] MT-CO2 mRNA [mitochondrial matrix] [Homo sapiens] [EntityWithAccessionedSequence:6793009] MT-CO3 mRNA [mitochondrial matrix] [Homo sapiens] [EntityWithAccessionedSequence:6792969] MT-CYB mRNA [mitochondrial matrix] [Homo sapiens] [EntityWithAccessionedSequence:6793007] MT-ND1 mRNA [mitochondrial matrix] [Homo sapiens] [EntityWithAccessionedSequence:6792988] MT-ND2 mRNA [mitochondrial matrix] [Homo sapiens] [EntityWithAccessionedSequence:6792965] MT-ND3 mRNA [mitochondrial matrix] [Homo sapiens] [EntityWithAccessionedSequence:6793019] MT-ND4 mRNA [mitochondrial matrix] [Homo sapiens] [EntityWithAccessionedSequence:6792983] MT-ND4L mRNA [mitochondrial matrix] [Homo sapiens] [EntityWithAccessionedSequence:6793008] MT-ND5 mRNA [mitochondrial matrix] [Homo sapiens] [EntityWithAccessionedSequence:6792992] pre-MT-TD [mitochondrial matrix] [Homo sapiens] [EntityWithAccessionedSequence:6792972] pre-MT-TF [mitochondrial matrix] [Homo sapiens] [EntityWithAccessionedSequence:6792985] pre-MT-TG [mitochondrial matrix] [Homo sapiens] [EntityWithAccessionedSequence:6792978] pre-MT-TH [mitochondrial matrix] [Homo sapiens] [EntityWithAccessionedSequence:6792999] pre-MT-TI [mitochondrial matrix] [Homo sapiens] [EntityWithAccessionedSequence:6792987] pre-MT-TK [mitochondrial matrix] [Homo sapiens] [EntityWithAccessionedSequence:6792970] pre-MT-TL1 [mitochondrial matrix] [Homo sapiens] [EntityWithAccessionedSequence:6793004] pre-MT-TL2 [mitochondrial matrix] [Homo sapiens] [EntityWithAccessionedSequence:6793015] pre-MT-TM [mitochondrial matrix] [Homo sapiens] [EntityWithAccessionedSequence:6792968] pre-MT-TR [mitochondrial matrix] [Homo sapiens] [EntityWithAccessionedSequence:6793000] pre-MT-TS2 [mitochondrial matrix] [Homo sapiens] [EntityWithAccessionedSequence:6792981] pre-MT-TT [mitochondrial matrix] [Homo sapiens] [EntityWithAccessionedSequence:6792986] pre-MT-TV [mitochondrial matrix] [Homo sapiens] [EntityWithAccessionedSequence:6792973] pre-MT-TW [mitochondrial matrix] [Homo sapiens]
precedingEvent
regulatedBy [Requirement:9685230] Requirement by 'mtRNase P [mitochondrial matrix]'
regulationReference [RegulationReference:9685232] Requirement by 'mtRNase P [mitochondrial matrix]' RNase P without RNA: identification and functional reconstitution of the human mitochondrial tRNA processing enzyme
releaseDate 2015-12-15
releaseStatus NEW
reviewed [InstanceEdit:6790491] Levinger, Louis, 2015-08-10 [InstanceEdit:6792690] Motorin, Yuri, 2015-08-25 [InstanceEdit:6806693] Jarrous, Nayef, 2015-10-24 [InstanceEdit:6808651] Powell, Christopher A, 2015-11-07 [InstanceEdit:6810571] Suzuki, Tsutomu, 2015-11-18
reviewStatus [ReviewStatus:9821382] five stars
species [Species:48887] Homo sapiens
stableIdentifier [StableIdentifier:6812614] R-HSA-6785722.6
summation [Summation:6786873] RNase P, ELAC2, and additional unknown nucleases cleave H st...
(hasEvent) [Pathway:6785470] tRNA processing in the mitochondrion [Homo sapiens] [Pathway:8868766] rRNA processing in the mitochondrion [Homo sapiens]
(precedingEvent) [Reaction:9936243] RPUSD4 in the mitochondrial pseudouridylation module transforms uridine-1397 to pseudouridine-1397 in mitochondrial 16S rRNA [Homo sapiens] [Reaction:9936260] RPUSD4 isomerizes uridine-39 of mitochondrial tRNA(Phe) (MT-TF) to pseudouridine [Homo sapiens] [Reaction:9936287] NSUN3 5-methylates cytosine-34 of mitochondrial tRNA(Met) (MT-TM) [Homo sapiens] [Reaction:9936998] MTPAP polyadenylates mitochondrial mRNA [Homo sapiens] [Reaction:9937369] RPUSD3,TRUB2 pseudouridylates MT-CO1,MT-CO3 mRNA [Homo sapiens] [Reaction:9937508] TRMT61B methylates adenosine-1472 of MT-CO1 mRNA [Homo sapiens] [Reaction:9937512] TRMT61B methylates adenosine-371 of MT-ND1 mRNA [Homo sapiens] [Reaction:9937518] TRMT61B methylates adenosine-333, adenosine-458, and adenosine-707 of MT-CO3 mRNA [Homo sapiens] [Reaction:9937521] TRMT61B methylates adenosine-185 of MT-CO2 mRNA [Homo sapiens] [Reaction:9937529] TRMT61B methylates adenosine-616 and adenosine-617 of MT-CYB mRNA [Homo sapiens]
(updatedInstance) [_UpdateTracker:9777035] Update Tracker - [BlackBoxEvent:6785722] Mitochondrial RNase P (mtRNase P) cleaves the 5' ends of pre-tRNAs and ELAC2 (RNase Z) cleaves the 3' ends of pre-tRNAs in the H strand transcript - v73:[addRegulator, removeCatalystActivity]
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