Skip Header

You are using a version of browser that may not display all the features of this website. Please consider upgrading your browser.
Protein

Mitochondrial ribonuclease P protein 3

Gene

KIAA0391

Organism
Homo sapiens (Human)
Status
Reviewed-Annotation score: Annotation score: 4 out of 5-Experimental evidence at protein leveli

Functioni

Functions in mitochondrial tRNA maturation. Part of mitochondrial ribonuclease P, an enzyme composed of MRPP1/TRMT10C, MRPP2/HSD17B10 and MRPP3/KIAA0391, which cleaves tRNA molecules in their 5'-ends.1 Publication

GO - Molecular functioni

GO - Biological processi

Complete GO annotation...

Keywords - Biological processi

tRNA processing

Enzyme and pathway databases

BioCyciZFISH:HS12429-MONOMER.
ReactomeiR-HSA-6785470. tRNA processing in the mitochondrion.
R-HSA-8868766. rRNA processing in the mitochondrion.

Names & Taxonomyi

Protein namesi
Recommended name:
Mitochondrial ribonuclease P protein 3
Short name:
Mitochondrial RNase P protein 3
Gene namesi
Name:KIAA0391
Synonyms:MRPP3
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
Proteomesi
  • UP000005640 Componenti: Chromosome 14

Organism-specific databases

HGNCiHGNC:19958. KIAA0391.

Subcellular locationi

GO - Cellular componenti

Complete GO annotation...

Keywords - Cellular componenti

Mitochondrion

Pathology & Biotechi

Organism-specific databases

DisGeNETi9692.
OpenTargetsiENSG00000100890.
ENSG00000258790.
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 protein 3Add BLAST538

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Modified residuei98PhosphoserineCombined sources1

Keywords - PTMi

Phosphoprotein

Proteomic databases

EPDiO15091.
MaxQBiO15091.
PaxDbiO15091.
PeptideAtlasiO15091.
PRIDEiO15091.

PTM databases

iPTMnetiO15091.
PhosphoSitePlusiO15091.

Expressioni

Gene expression databases

BgeeiENSG00000100890.
CleanExiHS_KIAA0391.
ExpressionAtlasiO15091. baseline and differential.
GenevisibleiO15091. HS.

Organism-specific databases

HPAiHPA020459.

Interactioni

Subunit structurei

Interacts with MRPP1/TRMT10C and MRPP2/HSD17B10.1 Publication

Protein-protein interaction databases

BioGridi115044. 22 interactors.
IntActiO15091. 4 interactors.
MINTiMINT-8051869.
STRINGi9606.ENSP00000454657.

Structurei

Secondary structure

1583
Legend: HelixTurnBeta strandPDB Structure known for this area
Show more details
Feature keyPosition(s)DescriptionActionsGraphical viewLength
Helixi213 – 220Combined sources8
Turni224 – 226Combined sources3
Helixi227 – 237Combined sources11
Helixi244 – 256Combined sources13
Helixi260 – 272Combined sources13
Helixi279 – 289Combined sources11
Helixi295 – 310Combined sources16
Helixi317 – 329Combined sources13
Beta strandi335 – 340Combined sources6
Beta strandi346 – 348Combined sources3
Turni349 – 351Combined sources3
Beta strandi357 – 359Combined sources3
Helixi362 – 376Combined sources15
Helixi388 – 399Combined sources12
Beta strandi405 – 409Combined sources5
Helixi410 – 413Combined sources4
Helixi423 – 433Combined sources11
Helixi434 – 436Combined sources3
Beta strandi440 – 445Combined sources6
Helixi446 – 449Combined sources4
Helixi460 – 466Combined sources7
Beta strandi467 – 472Combined sources6
Helixi481 – 489Combined sources9
Beta strandi494 – 498Combined sources5
Helixi511 – 524Combined sources14
Beta strandi553 – 561Combined sources9
Beta strandi575 – 581Combined sources7

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...

Family & Domainsi

Keywords - Domaini

Transit peptide

Phylogenomic databases

eggNOGiENOG410IE4T. Eukaryota.
ENOG4111GT9. LUCA.
GeneTreeiENSGT00390000002201.
HOVERGENiHBG099441.
InParanoidiO15091.
KOiK17655.
OMAiCVIDGLN.
OrthoDBiEOG091G0JTR.
PhylomeDBiO15091.
TreeFamiTF324726.

Family and domain databases

InterProiIPR033495. MRPP3.
IPR031595. PRORP_C.
[Graphical view]
PANTHERiPTHR13547:SF1. PTHR13547:SF1. 1 hit.
PfamiPF16953. PRORP. 1 hit.
[Graphical view]

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

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

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 → S.2 PublicationsCorresponds to variant rs11156878dbSNPEnsembl.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. [O15091-1]
XP_011535712.1. XM_011537410.2. [O15091-1]
XP_016877324.1. XM_017021835.1. [O15091-1]
XP_016877325.1. XM_017021836.1. [O15091-2]
XP_016877326.1. XM_017021837.1. [O15091-2]
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

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. [O15091-1]
XP_011535712.1. XM_011537410.2. [O15091-1]
XP_016877324.1. XM_017021835.1. [O15091-1]
XP_016877325.1. XM_017021836.1. [O15091-2]
XP_016877326.1. XM_017021837.1. [O15091-2]
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. 22 interactors.
IntActiO15091. 4 interactors.
MINTiMINT-8051869.
STRINGi9606.ENSP00000454657.

PTM databases

iPTMnetiO15091.
PhosphoSitePlusiO15091.

Polymorphism and mutation databases

BioMutaiKIAA0391.

Proteomic databases

EPDiO15091.
MaxQBiO15091.
PaxDbiO15091.
PeptideAtlasiO15091.
PRIDEiO15091.

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.
GeneCardsiKIAA0391.
H-InvDBHIX0018405.
HIX0079614.
HGNCiHGNC:19958. KIAA0391.
HPAiHPA020459.
MIMi609947. gene.
neXtProtiNX_O15091.
OpenTargetsiENSG00000100890.
ENSG00000258790.
PharmGKBiPA134879499.
HUGEiSearch...
GenAtlasiSearch...

Phylogenomic databases

eggNOGiENOG410IE4T. Eukaryota.
ENOG4111GT9. LUCA.
GeneTreeiENSGT00390000002201.
HOVERGENiHBG099441.
InParanoidiO15091.
KOiK17655.
OMAiCVIDGLN.
OrthoDBiEOG091G0JTR.
PhylomeDBiO15091.
TreeFamiTF324726.

Enzyme and pathway databases

BioCyciZFISH:HS12429-MONOMER.
ReactomeiR-HSA-6785470. tRNA processing in the mitochondrion.
R-HSA-8868766. rRNA processing in the mitochondrion.

Miscellaneous databases

ChiTaRSiKIAA0391. human.
GenomeRNAii9692.
PROiO15091.
SOURCEiSearch...

Gene expression databases

BgeeiENSG00000100890.
CleanExiHS_KIAA0391.
ExpressionAtlasiO15091. baseline and differential.
GenevisibleiO15091. HS.

Family and domain databases

InterProiIPR033495. MRPP3.
IPR031595. PRORP_C.
[Graphical view]
PANTHERiPTHR13547:SF1. PTHR13547:SF1. 1 hit.
PfamiPF16953. PRORP. 1 hit.
[Graphical view]
ProtoNetiSearch...

Entry informationi

Entry nameiMRRP3_HUMAN
AccessioniPrimary (citable) accession number: O15091
Secondary accession number(s): B4DXD9
, B4E0S8, B4E211, C4AM93, D3DS99, D3DSA1, Q86SZ4, Q86YB5, Q8N5L5
Entry historyi
Integrated into UniProtKB/Swiss-Prot: July 15, 1998
Last sequence update: January 20, 2009
Last modified: November 2, 2016
This is version 120 of the entry and version 2 of the sequence. [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. Human chromosome 14
    Human chromosome 14: entries, gene names and cross-references to MIM
  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

Similar proteinsi

Links to similar proteins from the UniProt Reference Clusters (UniRef) at 100%, 90% and 50% sequence identity:
100%UniRef100 combines identical sequences and sub-fragments with 11 or more residues from any organism into one UniRef entry.
90%UniRef90 is built by clustering UniRef100 sequences that have at least 90% sequence identity to, and 80% overlap with, the longest sequence (a.k.a seed sequence).
50%UniRef50 is built by clustering UniRef90 seed sequences that have at least 50% sequence identity to, and 80% overlap with, the longest sequence in the cluster.