MRPL3

Protein identityUniProt · HPA

UniProt accession

P09001

Gene

MRPL3

Ensembl

ENSG00000114686

Chromosome

3

Canonical length

348 aa

Protein class

Disease related genes, Human disease related genes, Predicted intracellular proteins, Ribosomal proteins

OverviewNCBI Gene

Mammalian mitochondrial ribosomal proteins are encoded by nuclear genes and help in protein synthesis within the mitochondrion. Mitochondrial ribosomes (mitoribosomes) consist of a small 28S subunit and a large 39S subunit. They have an estimated 75% protein to rRNA composition compared to prokaryotic ribosomes, where this ratio is reversed. Another difference between mammalian mitoribosomes and prokaryotic ribosomes is that the latter contain a 5S rRNA. Among different species, the proteins comprising the mitoribosome differ greatly in sequence, and sometimes in biochemical properties, which prevents easy recognition by sequence homology. This gene encodes a 39S subunit protein that belongs to the L3P ribosomal protein family. A pseudogene corresponding to this gene is found on chromosome 13q. [provided by RefSeq, Jul 2008]

Canonical amino-acid sequenceUniProt

348 residues, UniProt reviewed canonical sequence.

>P09001|MRPL3
     1  MPGWRLLTQV GAQVLGRLGD GLGAALGPGN RTHIWLFVRG LHGKSGTWWD EHLSEENVPF
    61  IKQLVSDEDK AQLASKLCPL KDEPWPIHPW EPGSFRVGLI ALKLGMMPLW TKDGQKHVVT
   121  LLQVQDCHVL KYTSKENCNG KMATLSVGGK TVSRFRKATS ILEFYRELGL PPKQTVKIFN
   181  ITDNAAIKPG TPLYAAHFRP GQYVDVTAKT IGKGFQGVMK RWGFKGQPAT HGQTKTHRRP
   241  GAVATGDIGR VWPGTKMPGK MGNIYRTEYG LKVWRINTKH NIIYVNGSVP GHKNCLVKVK
   301  DSKLPAYKDL GKNLPFPTYF PDGDEEELPE DLYDENVCQP GAPSITFA

LocalizationUniProt · AlphaFold · HPA

Whether an antibody against MRPL3 can act on the native protein depends on physical access: surface and secreted proteins are reachable by circulating antibodies, intracellular proteins usually are not.

Antibody reachability

Intracellular

Secreted

No

Transmembrane segments

0

Mean surface accessibility (rSASA)

0.38

Highest tissue expression

76 nTPM

Expression across tissuesHPA

DiseaseUniProt · ClinVar · IEDB · PubMed

Four sources answering four different questions about MRPL3.

Disease | AllUniProt

Conditions MRPL3 is implicated in, by any mechanism.

• Combined oxidative phosphorylation deficiency 9 (COXPD9) MIM:614582

Disease | GeneticClinVar

5 pathogenic / likely-pathogenic of 171 ClinVar records.

Conditions with pathogenic or likely-pathogenic variants.

• Combined oxidative phosphorylation defect type 9
• Neonatal encephalopathy

Genetic constraint and essentialitygnomAD · DepMap

Does the body need this protein intact? Low LOEUF or a strong DepMap dependency means loss or blockade of the protein is likely to be felt.

gnomAD LOEUF (loss-of-function intolerance)

1.38

gnomAD pLI

0

gnomAD missense Z

-0.16

DepMap mean gene effect

-0.39

DepMap dependency class

selective

Cancer expressionTCGA

Across TCGA tumor cohorts, this protein is over-expressed in roughly 11% of surveyed tumor types (aggregate summary; per-cohort expression, alteration, and survival load in the interactive view).

OntologyGO

Biological processes

• mitochondrial translation
• translation

Molecular functions

• RNA binding
• structural constituent of ribosome

Cellular components

• mitochondrial inner membrane
• mitochondrial large ribosomal subunit
• mitochondrion

Protein domainsUniProt · Pfam · InterPro

• Large ribosomal subunit protein uL3
• Translation protein, beta-barrel domain superfamily
• Large ribosomal subunit protein uL3, conserved site
• Ribosomal protein L3
• Large ribosomal subunit protein uL3, bacteria/organella

KeywordsUniProt

• Mitochondrion
• Primary mitochondrial disease
• Ribonucleoprotein
• Ribosomal protein
• Transit peptide

InteractionsUniProt · HPA

Protein binding partners of MRPL3 in the human serome: UniProt-annotated complex subunits plus reported interactors. Each links to its own Seroatlas record.

• MRPL10
• MRPL12
• MRPL13
• MRPL18
• MRPL23
• MRPL28
• MRPL37
• MRPL42
• MRPL46
• MRPL50
• MRPL52
• RPUSD4