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Protein

Mitochondrial ribonuclease P catalytic subunit

Gene

KIAA0391

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

Functioni

Catalytic ribonuclease component of mitochondrial ribonuclease P, a complex composed of TRMT10C/MRPP1, HSD17B10/MRPP2 and MRPP3, which cleaves tRNA molecules in their 5'-ends (PubMed:18984158, PubMed:25953853). The presence of TRMT10C/MRPP1, HSD17B10/MRPP2 is required to catalyze tRNA molecules in their 5'-ends (PubMed:25953853).2 Publications

Catalytic activityi

Endonucleolytic cleavage of RNA, removing 5'-extranucleotides from tRNA precursor.1 Publication

Cofactori

Mg2+1 Publication, Mn2+1 PublicationNote: Binds 2 Mg2+ or Mg2+ ions per subunit.1 Publication

Sites

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Metal bindingi348ZincCombined sources1 Publication1
Metal bindingi351ZincCombined sources1 Publication1
Metal bindingi409Magnesium or manganese 1; catalytic1 Publication1
Metal bindingi478Magnesium or manganese 1; catalytic1 Publication1
Metal bindingi479Magnesium or manganese 2; catalytic1 Publication1
Metal bindingi499Magnesium or manganese 2; catalytic1 Publication1
Metal bindingi557Zinc; via tele nitrogenCombined sources1 Publication1
Metal bindingi578ZincCombined sources1 Publication1

GO - Molecular functioni

  • metal ion binding Source: UniProtKB-KW
  • ribonuclease P activity Source: UniProtKB

GO - Biological processi

  • mitochondrial tRNA 5'-end processing Source: UniProtKB
  • mitochondrial tRNA processing Source: Reactome
  • tRNA 5'-leader removal Source: GO_Central

Keywordsi

Molecular functionHydrolase, Nuclease
Biological processtRNA processing
LigandMagnesium, Manganese, Metal-binding

Enzyme and pathway databases

ReactomeiR-HSA-6785470 tRNA processing in the mitochondrion
R-HSA-6787450 tRNA modification in the mitochondrion
R-HSA-8868766 rRNA processing in the mitochondrion

Names & Taxonomyi

Protein namesi
Recommended name:
Mitochondrial ribonuclease P catalytic subunit (EC:3.1.26.51 Publication)
Alternative name(s):
Mitochondrial ribonuclease P protein 31 Publication
Short name:
Mitochondrial RNase P protein 31 Publication
Gene namesi
Name:KIAA0391Imported
Synonyms:MRPP31 Publication
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
Proteomesi
  • UP000005640 Componenti: Chromosome 14

Organism-specific databases

EuPathDBiHostDB:ENSG00000100890.15
HGNCiHGNC:19958 KIAA0391
MIMi609947 gene
neXtProtiNX_O15091

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

Mitochondrion

Pathology & Biotechi

Mutagenesis

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Mutagenesisi409D → N: Abolishes ribonuclease activity. 1 Publication1
Mutagenesisi478D → N: Abolishes ribonuclease activity. 1 Publication1
Mutagenesisi479D → N: Abolishes ribonuclease activity. 1 Publication1
Mutagenesisi480P → G: Does not affect ribonuclease activity. 1 Publication1
Mutagenesisi498R → D or N: Does not affect ribonuclease activity. 1 Publication1
Mutagenesisi499D → N: Abolishes ribonuclease activity. 1 Publication1
Mutagenesisi569S → A: Does not affect ribonuclease activity. 1 Publication1

Organism-specific databases

DisGeNETi9692
OpenTargetsiENSG00000100890
PharmGKBiPA134879499

Polymorphism and mutation databases

BioMutaiKIAA0391

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Transit peptidei1 – 45MitochondrionSequence analysisAdd BLAST45
ChainiPRO_000005074946 – 583Mitochondrial ribonuclease P catalytic subunitAdd BLAST538

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Modified residuei98PhosphoserineCombined sources1

Post-translational modificationi

Degraded by LONP1 following mitochondrial unfolded protein response, probably leading to inhibit translation in mitochondrion.1 Publication

Keywords - PTMi

Phosphoprotein

Proteomic databases

EPDiO15091
MaxQBiO15091
PaxDbiO15091
PeptideAtlasiO15091
PRIDEiO15091
ProteomicsDBi48444
48445 [O15091-2]
48446 [O15091-3]
48447 [O15091-4]

PTM databases

iPTMnetiO15091
PhosphoSitePlusiO15091

Expressioni

Inductioni

Down-regulated following mitochondrial unfolded protein response.1 Publication

Gene expression databases

BgeeiENSG00000100890 Expressed in 107 organ(s), highest expression level in leukocyte
CleanExiHS_KIAA0391
ExpressionAtlasiO15091 baseline and differential
GenevisibleiO15091 HS

Organism-specific databases

HPAiHPA020459

Interactioni

Subunit structurei

Catalytic component of mitochondrial ribonuclease P, a complex composed of TRMT10C/MRPP1, HSD17B10/MRPP2 and MRPP31 (PubMed:18984158).1 Publication

Protein-protein interaction databases

BioGridi115044, 31 interactors
IntActiO15091, 6 interactors
MINTiO15091
STRINGi9606.ENSP00000454657

Structurei

Secondary structure

1583
Legend: HelixTurnBeta strandPDB Structure known for this area
Show more details

3D structure databases

ProteinModelPortaliO15091
SMRiO15091
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Domains and Repeats

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Domaini342 – 578PRORPAdd BLAST237

Domaini

Displays a distorted and non-productive active site that probably switches to a fully productive state only upon association with TRMT10C/MRPP1, HSD17B10/MRPP2 and pre-tRNA substrate.1 Publication

Sequence similaritiesi

Belongs to the PPR family. P subfamily.Curated

Keywords - Domaini

Transit peptide

Phylogenomic databases

eggNOGiENOG410IE4T Eukaryota
ENOG4111GT9 LUCA
GeneTreeiENSGT00390000002201
HOVERGENiHBG099441
InParanoidiO15091
KOiK17655
OMAiHDDPFLL
OrthoDBiEOG091G0JTR
PhylomeDBiO15091
TreeFamiTF324726

Family and domain databases

Gene3Di1.25.40.10, 1 hit
InterProiView protein in InterPro
IPR033495 MRPP3
IPR031595 PRORP_C
IPR011990 TPR-like_helical_dom_sf
PANTHERiPTHR13547:SF1 PTHR13547:SF1, 1 hit
PfamiView protein in Pfam
PF16953 PRORP, 1 hit

Sequences (4+)i

Sequence statusi: Complete.

Sequence processingi: The displayed sequence is further processed into a mature form.

This entry describes 4 isoformsi produced by alternative splicing. AlignAdd to basket

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

Isoform 1 (identifier: O15091-1) [UniParc]FASTAAdd to basket

This isoform has been chosen as the 'canonical' sequence. All positional information in this entry refers to it. This is also the sequence that appears in the downloadable versions of the entry.

« Hide
        10         20         30         40         50
MTFYLFGIRS FPKLWKSPYL GLGPGHSYVS LFLADRCGIR NQQRLFSLKT
60 70 80 90 100
MSPQNTKATN LIAKARYLRK DEGSNKQVYS VPHFFLAGAA KERSQMNSQT
110 120 130 140 150
EDHALAPVRN TIQLPTQPLN SEEWDKLKED LKENTGKTSF ESWIISQMAG
160 170 180 190 200
CHSSIDVAKS LLAWVAAKNN GIVSYDLLVK YLYLCVFHMQ TSEVIDVFEI
210 220 230 240 250
MKARYKTLEP RGYSLLIRGL IHSDRWREAL LLLEDIKKVI TPSKKNYNDC
260 270 280 290 300
IQGALLHQDV NTAWNLYQEL LGHDIVPMLE TLKAFFDFGK DIKDDNYSNK
310 320 330 340 350
LLDILSYLRN NQLYPGESFA HSIKTWFESV PGKQWKGQFT TVRKSGQCSG
360 370 380 390 400
CGKTIESIQL SPEEYECLKG KIMRDVIDGG DQYRKTTPQE LKRFENFIKS
410 420 430 440 450
RPPFDVVIDG LNVAKMFPKV RESQLLLNVV SQLAKRNLRL LVLGRKHMLR
460 470 480 490 500
RSSQWSRDEM EEVQKQASCF FADDISEDDP FLLYATLHSG NHCRFITRDL
510 520 530 540 550
MRDHKACLPD AKTQRLFFKW QQGHQLAIVN RFPGSKLTFQ RILSYDTVVQ
560 570 580
TTGDSWHIPY DEDLVERCSC EVPTKWLCLH QKT
Length:583
Mass (Da):67,315
Last modified:January 20, 2009 - v2
Checksum:i6AB17E7E8820D818
GO
Isoform 2 (identifier: O15091-2) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     329-344: Missing.

Show »
Length:567
Mass (Da):65,486
Checksum:i67B870DEBE05F5CC
GO
Isoform 3 (identifier: O15091-3) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     1-372: Missing.

Show »
Length:211
Mass (Da):24,787
Checksum:iD8B8A46D9244A3BD
GO
Isoform 4 (identifier: O15091-4) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     1-95: Missing.

Note: No experimental confirmation available.
Show »
Length:488
Mass (Da):56,487
Checksum:iEF0D334CD9099547
GO

Computationally mapped potential isoform sequencesi

There are 4 potential isoforms mapped to this entry.BLASTAlignShow allAdd to basket
EntryEntry nameProtein names
Gene namesLengthAnnotation
A0A0A0MTQ0A0A0A0MTQ0_HUMAN
Mitochondrial ribonuclease P cataly...
KIAA0391
185Annotation score:
S4R3T4S4R3T4_HUMAN
Mitochondrial ribonuclease P cataly...
KIAA0391
23Annotation score:
S4R416S4R416_HUMAN
Mitochondrial ribonuclease P cataly...
KIAA0391
34Annotation score:
S4R3S1S4R3S1_HUMAN
Mitochondrial ribonuclease P cataly...
KIAA0391
19Annotation score:

Sequence cautioni

The sequence BAA20845 differs from that shown. Reason: Erroneous initiation.Curated

Experimental Info

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Sequence conflicti315P → L in BAG63351 (PubMed:14702039).Curated1
Sequence conflicti453S → F in BAG64540 (PubMed:14702039).Curated1

Natural variant

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_054212437N → S2 PublicationsCorresponds to variant dbSNP:rs11156878Ensembl.1

Alternative sequence

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Alternative sequenceiVSP_0362011 – 372Missing in isoform 3. 2 PublicationsAdd BLAST372
Alternative sequenceiVSP_0362021 – 95Missing in isoform 4. 1 PublicationAdd BLAST95
Alternative sequenceiVSP_036203329 – 344Missing in isoform 2. 2 PublicationsAdd BLAST16

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AB002389 mRNA Translation: BAA20845.2 Different initiation.
BX161394 mRNA Translation: CAD61881.1
BX161487 mRNA Translation: CAD61937.1
AK301931 mRNA Translation: BAG63351.1
AK303508 mRNA Translation: BAG64540.1
AK304066 mRNA Translation: BAG64973.1
AL121594 Genomic DNA No translation available.
CH471078 Genomic DNA Translation: EAW65878.1
CH471078 Genomic DNA Translation: EAW65882.1
CH471078 Genomic DNA Translation: EAW65879.1
CH471078 Genomic DNA Translation: EAW65880.1
CH471078 Genomic DNA Translation: EAW65881.1
CH471078 Genomic DNA Translation: EAW65883.1
CH471078 Genomic DNA Translation: EAW65884.1
BC032221 mRNA Translation: AAH32221.1
BC044580 mRNA Translation: AAH44580.1
CCDSiCCDS32063.1 [O15091-1]
CCDS58312.1 [O15091-2]
CCDS58313.1 [O15091-4]
CCDS58314.1 [O15091-3]
RefSeqiNP_001243607.1, NM_001256678.1 [O15091-2]
NP_001243608.1, NM_001256679.1 [O15091-4]
NP_001243609.1, NM_001256680.1 [O15091-3]
NP_001243610.1, NM_001256681.1 [O15091-3]
NP_055487.2, NM_014672.3 [O15091-1]
XP_005268294.1, XM_005268237.3 [O15091-1]
XP_011535711.1, XM_011537409.2
XP_011535712.1, XM_011537410.2 [O15091-1]
XP_016877324.1, XM_017021835.1
XP_016877325.1, XM_017021836.1 [O15091-2]
XP_016877326.1, XM_017021837.1
UniGeneiHs.458487
Hs.739309

Genome annotation databases

EnsembliENST00000250377; ENSP00000250377; ENSG00000100890 [O15091-2]
ENST00000321130; ENSP00000324697; ENSG00000100890 [O15091-3]
ENST00000534898; ENSP00000440915; ENSG00000100890 [O15091-1]
ENST00000603544; ENSP00000473856; ENSG00000100890 [O15091-2]
ENST00000604948; ENSP00000474620; ENSG00000100890 [O15091-4]
ENST00000605870; ENSP00000474299; ENSG00000100890 [O15091-3]
GeneIDi9692
KEGGihsa:9692
UCSCiuc001wsy.3 human [O15091-1]

Keywords - Coding sequence diversityi

Alternative splicing, Polymorphism

Similar proteinsi

Cross-referencesi

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AB002389 mRNA Translation: BAA20845.2 Different initiation.
BX161394 mRNA Translation: CAD61881.1
BX161487 mRNA Translation: CAD61937.1
AK301931 mRNA Translation: BAG63351.1
AK303508 mRNA Translation: BAG64540.1
AK304066 mRNA Translation: BAG64973.1
AL121594 Genomic DNA No translation available.
CH471078 Genomic DNA Translation: EAW65878.1
CH471078 Genomic DNA Translation: EAW65882.1
CH471078 Genomic DNA Translation: EAW65879.1
CH471078 Genomic DNA Translation: EAW65880.1
CH471078 Genomic DNA Translation: EAW65881.1
CH471078 Genomic DNA Translation: EAW65883.1
CH471078 Genomic DNA Translation: EAW65884.1
BC032221 mRNA Translation: AAH32221.1
BC044580 mRNA Translation: AAH44580.1
CCDSiCCDS32063.1 [O15091-1]
CCDS58312.1 [O15091-2]
CCDS58313.1 [O15091-4]
CCDS58314.1 [O15091-3]
RefSeqiNP_001243607.1, NM_001256678.1 [O15091-2]
NP_001243608.1, NM_001256679.1 [O15091-4]
NP_001243609.1, NM_001256680.1 [O15091-3]
NP_001243610.1, NM_001256681.1 [O15091-3]
NP_055487.2, NM_014672.3 [O15091-1]
XP_005268294.1, XM_005268237.3 [O15091-1]
XP_011535711.1, XM_011537409.2
XP_011535712.1, XM_011537410.2 [O15091-1]
XP_016877324.1, XM_017021835.1
XP_016877325.1, XM_017021836.1 [O15091-2]
XP_016877326.1, XM_017021837.1
UniGeneiHs.458487
Hs.739309

3D structure databases

Select the link destinations:
PDBei
RCSB PDBi
PDBji
Links Updated
PDB entryMethodResolution (Å)ChainPositionsPDBsum
4ROUX-ray2.71A/B274-583[»]
4XGLX-ray1.80A207-583[»]
4XGMX-ray1.98A207-583[»]
ProteinModelPortaliO15091
SMRiO15091
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

BioGridi115044, 31 interactors
IntActiO15091, 6 interactors
MINTiO15091
STRINGi9606.ENSP00000454657

PTM databases

iPTMnetiO15091
PhosphoSitePlusiO15091

Polymorphism and mutation databases

BioMutaiKIAA0391

Proteomic databases

EPDiO15091
MaxQBiO15091
PaxDbiO15091
PeptideAtlasiO15091
PRIDEiO15091
ProteomicsDBi48444
48445 [O15091-2]
48446 [O15091-3]
48447 [O15091-4]

Protocols and materials databases

Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENST00000250377; ENSP00000250377; ENSG00000100890 [O15091-2]
ENST00000321130; ENSP00000324697; ENSG00000100890 [O15091-3]
ENST00000534898; ENSP00000440915; ENSG00000100890 [O15091-1]
ENST00000603544; ENSP00000473856; ENSG00000100890 [O15091-2]
ENST00000604948; ENSP00000474620; ENSG00000100890 [O15091-4]
ENST00000605870; ENSP00000474299; ENSG00000100890 [O15091-3]
GeneIDi9692
KEGGihsa:9692
UCSCiuc001wsy.3 human [O15091-1]

Organism-specific databases

CTDi9692
DisGeNETi9692
EuPathDBiHostDB:ENSG00000100890.15
GeneCardsiKIAA0391
H-InvDBiHIX0018405
HIX0079614
HGNCiHGNC:19958 KIAA0391
HPAiHPA020459
MIMi609947 gene
neXtProtiNX_O15091
OpenTargetsiENSG00000100890
PharmGKBiPA134879499
HUGEiSearch...
GenAtlasiSearch...

Phylogenomic databases

eggNOGiENOG410IE4T Eukaryota
ENOG4111GT9 LUCA
GeneTreeiENSGT00390000002201
HOVERGENiHBG099441
InParanoidiO15091
KOiK17655
OMAiHDDPFLL
OrthoDBiEOG091G0JTR
PhylomeDBiO15091
TreeFamiTF324726

Enzyme and pathway databases

ReactomeiR-HSA-6785470 tRNA processing in the mitochondrion
R-HSA-6787450 tRNA modification in the mitochondrion
R-HSA-8868766 rRNA processing in the mitochondrion

Miscellaneous databases

ChiTaRSiKIAA0391 human
GenomeRNAii9692
PROiPR:O15091
SOURCEiSearch...

Gene expression databases

BgeeiENSG00000100890 Expressed in 107 organ(s), highest expression level in leukocyte
CleanExiHS_KIAA0391
ExpressionAtlasiO15091 baseline and differential
GenevisibleiO15091 HS

Family and domain databases

Gene3Di1.25.40.10, 1 hit
InterProiView protein in InterPro
IPR033495 MRPP3
IPR031595 PRORP_C
IPR011990 TPR-like_helical_dom_sf
PANTHERiPTHR13547:SF1 PTHR13547:SF1, 1 hit
PfamiView protein in Pfam
PF16953 PRORP, 1 hit
ProtoNetiSearch...

Entry informationi

Entry nameiMRPP3_HUMAN
AccessioniPrimary (citable) accession number: O15091
Secondary accession number(s): B4DXD9
, B4E0S8, B4E211, C4AM93, D3DS99, D3DSA1, Q86SZ4, Q86YB5, Q8N5L5
Entry historyiIntegrated into UniProtKB/Swiss-Prot: July 15, 1998
Last sequence update: January 20, 2009
Last modified: November 7, 2018
This is version 138 of the entry and version 2 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

3D-structure, Complete proteome, Reference proteome

Documents

  1. SIMILARITY comments
    Index of protein domains and families
  2. Human entries with polymorphisms or disease mutations
    List of human entries with polymorphisms or disease mutations
  3. Human polymorphisms and disease mutations
    Index of human polymorphisms and disease mutations
  4. MIM cross-references
    Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot
  5. PDB cross-references
    Index of Protein Data Bank (PDB) cross-references
  6. Human chromosome 14
    Human chromosome 14: entries, gene names and cross-references to MIM
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