MRPL10

protein-coding

mitochondrial ribosomal protein L10, the group of Large subunit mitochondrial ribosomal proteins|Mitochondrial ribosomal proteins

Gene summary

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. Sequence analysis identified three transcript variants that encode two different isoforms. A pseudogene corresponding to this gene is found on chromosome 5q. [provided by RefSeq, Nov 2010]

MANE transcript

NM_145255.4

Inheritance

—

ClinVar variants

U:36B:3

Top phenotype

MRPL10-related disorder

gnomAD constraint

pLI —, LoF o/e 0.33

Max pathogenic AF

—

Genomic locus

chr17:47,823,272-47,831,541

External References & Databases

Transcript & Mutation Effects

hg38 • Region Span ~8.3 kb
Loading mutation effect viewer...

GenCC Phenotypes & Disease Associations

Curated gene-disease validity assertions and inheritance patterns

No GenCC data available for this gene.

ClinVar Associated Diseases & Phenotypes

Reported disease conditions connected to MRPL10 variants in ClinVar

2 Conditions Cataloged
not specifiedTotal: 34
MRPL10-related disorderTotal: 2

ClinVar Variants & Pathogenicity by Type

Statistics based on transcript NM_145255.4. Only rare variants are included.

We include only reliable ClinVar variants with their consequences to MANE Select, MANE Plus Clinical transcripts, or transcripts with TSL equal to 1. Click a count to filter the variants table below.

Slight differences between displayed counts and ClinVar may occur due to a different transcript/consequence chosen by our variant effect predictor, or because we classify Benign/Likely benign as Likely benign and Pathogenic/Likely pathogenic as Likely pathogenic.

EffectPLPVUSLBBSum
synonymous
2
clinvar
2
missense
36
clinvar
1
clinvar
37
nonsense
0
start loss
0
frameshift
0
splice donor/acceptor (+/-2bp)
0
Total00363039
Loading ClinVar variants...

Transcript Catalog

Annotated transcript isoforms across RefSeq and Ensembl databases

Showing 4 of 20 transcripts
Transcript IDSourceProtein IDCoding ExonsMANE Status
NM_145255.4RefSeqNP_660298.25MANE Select
ENST00000351111.7EnsemblENSP00000324100.45MANE Select
NM_148887.3RefSeqNP_683685.15Standard
ENST00000290208.11EnsemblENSP00000290208.75Standard

Gene Ontology (GO) Annotations & Pathways

Biological processes, molecular functions, cellular components and pathways

Pathways ConsensusPathDB

  • Ribosome - Homo sapiens (human)
  • Mitochondrial translation initiation
  • Translation
  • Metabolism of proteins
  • Mitochondrial translation elongation

Biological Process

  • translation
  • ribosome biogenesis
  • mitochondrial translational elongation
  • mitochondrial translational termination

Molecular Function

  • RNA binding
  • structural constituent of ribosome
  • protein binding

Cellular Component

  • nucleoplasm
  • mitochondrion
  • mitochondrial inner membrane
  • mitochondrial large ribosomal subunit
  • large ribosomal subunit

gnomAD Constraint

Source: gnomAD

Gene type
protein_coding
Transcript type
protein_coding
Transcript
ENST00000290208
Coding exons
5
Length
8263
Individuals with no LoFs
125638
Homozygous LoFs
0
Heterozygous LoFs
14
Defined
125652
p
0.0000557
Z-ScoreObservedExpectedObserved/ExpectedMutation RateTotal Possible
Missense0.08331401430.9800.000008551734
Missense (PolyPhen)2634.6850.7496429
Synonymous0.4575458.40.9240.00000313581
Loss of Function1.8639.020.3335.52e-7108

Population LoF Frequency

PopulationCAFp-valueStatus
African00.0absent
Latino/Admixed American0.0000590.000058rare
Ashkenazi Jewish00.0absent
East Asian00.0absent
Finnish00.0absent
European (Non-Finnish)0.0000730.000070rare
South Asian0.0000660.000065rare
Other0.000340.00033rare

dbNSFP Gene Annotations

Source: dbNSFP

Recessive Scores

pRec
0.0833

Intolerance Scores

LoFtool
0.392
RVIS EVS
-0.1
RVIS percentile EVS
46.49

Essentials

Essential (CRISPR)
E
Essential (CRISPR2)
S
Essential (gene trap)
E
Indispensability pred.
E
Indispensability score
0.987

Gene Damage Prediction

AllRecessiveDominant
MendelianMediumMediumMedium
Primary ImmunodeficiencyMediumMediumMedium
CancerMediumMediumMedium
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