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UniProtKB - P09001 (RM03_HUMAN)

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Protein

39S ribosomal protein L3, mitochondrial

Gene

MRPL3

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

Functioni

GO - Molecular functioni

View the complete GO annotation on QuickGO ...

GO - Biological processi

  • mitochondrial translational elongation Source: Reactome
  • mitochondrial translational termination Source: Reactome
  • translation Source: ProtInc
View the complete GO annotation on QuickGO ...

Keywordsi

Molecular functionRibonucleoprotein, Ribosomal protein

Enzyme and pathway databases

ReactomeiR-HSA-5368286 Mitochondrial translation initiation
R-HSA-5389840 Mitochondrial translation elongation
R-HSA-5419276 Mitochondrial translation termination

Names & Taxonomyi

Protein namesi
Recommended name:
39S ribosomal protein L3, mitochondrial
Short name:
L3mt
Short name:
MRP-L3
Alternative name(s):
Mitochondrial large ribosomal subunit protein uL3m1 Publication
Gene namesi
Name:MRPL3
Synonyms:MRL3, RPML3
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryota › Metazoa › Chordata › Craniata › Vertebrata › Euteleostomi › Mammalia › Eutheria › Euarchontoglires › Primates › Haplorrhini › Catarrhini › Hominidae › Homo
Proteomesi
  • UP000005640 Componenti: Chromosome 3

Organism-specific databases

EuPathDBiHostDB:ENSG00000114686.8
HGNCiHGNC:10379 MRPL3
MIMi607118 gene
neXtProtiNX_P09001

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

Involvement in diseasei

Combined oxidative phosphorylation deficiency 9 (COXPD9)1 Publication
The disease is caused by mutations affecting the gene represented in this entry.
Disease descriptionA mitochondrial disease characterized by failure to thrive, poor feeding, hypertrophic cardiomyopathy, hepatomegaly, and psychomotor retardation. Death in infancy has been observed in some cases.
See also OMIM:614582
Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_066676317P → R in COXPD9. 1 PublicationCorresponds to variant dbSNP:rs387906962EnsemblClinVar.1

Keywords - Diseasei

Disease mutation, Primary mitochondrial disease

Organism-specific databases

DisGeNETi11222
MalaCardsiMRPL3
MIMi614582 phenotype
OpenTargetsiENSG00000114686
Orphaneti319509 Combined oxidative phosphorylation defect type 9
PharmGKBiPA30960

Polymorphism and mutation databases

BioMutaiMRPL3

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Transit peptidei1 – 40MitochondrionCombined sourcesAdd BLAST40
ChainiPRO_000007725341 – 34839S ribosomal protein L3, mitochondrialAdd BLAST308

Proteomic databases

EPDiP09001
MaxQBiP09001
PaxDbiP09001
PeptideAtlasiP09001
PRIDEiP09001
ProteomicsDBi52183

PTM databases

iPTMnetiP09001
PhosphoSitePlusiP09001
SwissPalmiP09001

Expressioni

Gene expression databases

BgeeiENSG00000114686
CleanExiHS_MRPL3
ExpressionAtlasiP09001 baseline and differential
GenevisibleiP09001 HS

Organism-specific databases

HPAiHPA043665
HPA060132

Interactioni

Subunit structurei

Component of the mitochondrial large ribosomal subunit (mt-LSU). Mature mammalian 55S mitochondrial ribosomes consist of a small (28S) and a large (39S) subunit. The 28S small subunit contains a 12S ribosomal RNA (12S mt-rRNA) and 30 different proteins. The 39S large subunit contains a 16S rRNA (16S mt-rRNA), a copy of mitochondrial valine transfer RNA (mt-tRNA(Val)), which plays an integral structural role, and 52 different proteins.2 Publications

Protein-protein interaction databases

BioGridi116390, 64 interactors
CORUMiP09001
IntActiP09001, 29 interactors
MINTiP09001
STRINGi9606.ENSP00000264995

Structurei

Secondary structure

1348
Legend: HelixTurnBeta strandPDB Structure known for this area
Show more details
Feature keyPosition(s)DescriptionActionsGraphical viewLength
Turni49 – 52Combined sources4
Turni55 – 57Combined sources3
Helixi58 – 75Combined sources18
Beta strandi79 – 82Combined sources4
Beta strandi99 – 111Combined sources13
Beta strandi116 – 123Combined sources8
Beta strandi126 – 133Combined sources8
Helixi135 – 138Combined sources4
Beta strandi140 – 151Combined sources12
Helixi154 – 156Combined sources3
Helixi159 – 168Combined sources10
Beta strandi173 – 181Combined sources9
Beta strandi183 – 185Combined sources3
Helixi195 – 197Combined sources3
Beta strandi203 – 209Combined sources7
Beta strandi214 – 216Combined sources3
Helixi218 – 221Combined sources4
Beta strandi229 – 232Combined sources4
Beta strandi237 – 239Combined sources3
Beta strandi246 – 248Combined sources3
Beta strandi258 – 262Combined sources5
Beta strandi264 – 277Combined sources14
Turni278 – 281Combined sources4
Beta strandi282 – 287Combined sources6
Beta strandi296 – 301Combined sources6
Helixi305 – 307Combined sources3
Beta strandi312 – 314Combined sources3
Helixi321 – 323Combined sources3
Beta strandi330 – 333Combined sources4

3D structure databases

ProteinModelPortaliP09001
SMRiP09001
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Sequence similaritiesi

Belongs to the universal ribosomal protein uL3 family.Curated

Keywords - Domaini

Transit peptide

Phylogenomic databases

eggNOGiKOG3141 Eukaryota
COG0087 LUCA
GeneTreeiENSGT00390000011422
HOGENOMiHOG000100369
HOVERGENiHBG002294
InParanoidiP09001
KOiK02906
PhylomeDBiP09001
TreeFamiTF105634

Family and domain databases

InterProiView protein in InterPro
IPR000597 Ribosomal_L3
IPR019927 Ribosomal_L3_bac/org-type
IPR019926 Ribosomal_L3_CS
IPR009000 Transl_B-barrel_sf
PANTHERiPTHR11229 PTHR11229, 1 hit
PfamiView protein in Pfam
PF00297 Ribosomal_L3, 1 hit
SUPFAMiSSF50447 SSF50447, 1 hit
TIGRFAMsiTIGR03625 L3_bact, 1 hit
PROSITEiView protein in PROSITE
PS00474 RIBOSOMAL_L3, 1 hit

Sequencei

Sequence statusi: Complete.

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

P09001-1 [UniParc]FASTAAdd to basket

« Hide

        10         20         30         40         50
MPGWRLLTQV GAQVLGRLGD GLGAALGPGN RTHIWLFVRG LHGKSGTWWD 
        60         70         80         90        100
EHLSEENVPF IKQLVSDEDK AQLASKLCPL KDEPWPIHPW EPGSFRVGLI 
       110        120        130        140        150
ALKLGMMPLW TKDGQKHVVT LLQVQDCHVL KYTSKENCNG KMATLSVGGK 
       160        170        180        190        200
TVSRFRKATS ILEFYRELGL PPKQTVKIFN ITDNAAIKPG TPLYAAHFRP 
       210        220        230        240        250
GQYVDVTAKT IGKGFQGVMK RWGFKGQPAT HGQTKTHRRP GAVATGDIGR 
       260        270        280        290        300
VWPGTKMPGK MGNIYRTEYG LKVWRINTKH NIIYVNGSVP GHKNCLVKVK 
       310        320        330        340 
DSKLPAYKDL GKNLPFPTYF PDGDEEELPE DLYDENVCQP GAPSITFA
Length:348
Mass (Da):38,633
Last modified:November 1, 1988 - v1
Checksum:i6DE6B5BD6BD72E32
GO

Natural variant

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_020108261M → T. Corresponds to variant dbSNP:rs2291381EnsemblClinVar.1
Natural variantiVAR_066676317P → R in COXPD9. 1 PublicationCorresponds to variant dbSNP:rs387906962EnsemblClinVar.1

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
X06323 mRNA Translation: CAA29639.1
CR456720 mRNA Translation: CAG33001.1
CH471052 Genomic DNA Translation: EAW79209.1
BC003375 mRNA Translation: AAH03375.1
CCDSiCCDS3071.1
PIRiA27294 R5HUL3
RefSeqiNP_009139.1, NM_007208.3
UniGeneiHs.205163

Genome annotation databases

EnsembliENST00000264995; ENSP00000264995; ENSG00000114686
GeneIDi11222
KEGGihsa:11222
UCSCiuc003eoh.4 human

Keywords - Coding sequence diversityi

Polymorphism

Similar proteinsi

Entry informationi

Entry nameiRM03_HUMAN
AccessioniPrimary (citable) accession number: P09001
Secondary accession number(s): Q6IBT2
Entry historyiIntegrated into UniProtKB/Swiss-Prot: November 1, 1988
Last sequence update: November 1, 1988
Last modified: June 20, 2018
This is version 167 of the entry and version 1 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. Human chromosome 3
    Human chromosome 3: 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
  6. Ribosomal proteins
    Ribosomal proteins families and list of entries
  7. SIMILARITY comments
    Index of protein domains and families

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