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10 sources retrieved · Most recent: April 2026 · Index updated 14 days ago
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MRM3
mitochondrial rRNA methyltransferase 3
Chromosome 17 · 17p13.3
NCBI Gene: 55178Ensembl: ENSG00000171861.12HGNC: HGNC:18485UniProt: Q9HC36
85PubMed Papers
20Diseases
0Drugs
0Pathogenic Variants
FUNCTIONAL ROLE
Hub Gene
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
rRNA processingprotein bindingmitochondrionmitochondrial matrixmaturity-onset diabetes of the young type 3MODY3-hydroxy-3-methylglutaryl-CoA synthase deficiencyexercise-induced hyperinsulinism
✦AI Summary

MRM3 (mitochondrial rRNA methyltransferase 3) is an S-adenosyl-L-methionine-dependent 2'-O-ribose methyltransferase that catalyzes 2'-O-methylguanosine formation at position 1370 (Gm1370) in the 16S mitochondrial large subunit ribosomal RNA (mtLSU rRNA) 1. This modification occurs in the peptidyl transferase domain and is interdependent with MRM2-mediated Um1369 methylation 2. MRM3 functions as a critical assembly factor for late-stage human 39S mitoribosome biogenesis, maintaining proper 16S rRNA conformation during this process 3. Depletion of MRM3 in human cells causes respiratory incompetence through diminished mitochondrial translation and aberrant assembly of the mt-LSU 1. During human cytomegalovirus infection, MRM3 is highly upregulated as part of the viral manipulation of mitochondrial biogenesis; knockdown of MRM3 abolishes virus-mediated increases in mitochondrially encoded proteins and significantly impairs viral replication 4. A recent Mendelian randomization study identified MRM3 as a potential risk factor for sepsis and sepsis-related mortality 5. Thus, MRM3 represents an essential regulator of mitochondrial protein synthesis with implications in metabolic homeostasis, viral pathogenesis, and sepsis pathophysiology.

Sources cited
1
MRM3 catalyzes 2'-O-methylation of 16S rRNA and is essential for mtLSU biogenesis and mitochondrial translation
PMID: 25009282
2
MRM3 modifications are interdependent with MRM2 and MRM3 acts as an assembly factor maintaining immature rRNA conformations
PMID: 35177605
3
MRM3 is identified as a late-stage assembly factor in human 39S pre-mitoribosome cryo-EM structures
PMID: 34315873
4
MRM3 is upregulated during HCMV infection and is required for viral-induced mitochondrial biogenesis and viral replication
PMID: 27025248
5
Mendelian randomization analysis identifies MRM3 as a risk factor for sepsis
PMID: 41088638
Disease Associationsⓘ20
maturity-onset diabetes of the young type 3Open Targets
0.04Suggestive
MODYOpen Targets
0.04Suggestive
3-hydroxy-3-methylglutaryl-CoA synthase deficiencyOpen Targets
0.04Suggestive
exercise-induced hyperinsulinismOpen Targets
0.04Suggestive
Glycogen storage disease due to hepatic glycogen synthase deficiencyOpen Targets
0.04Suggestive
glycogen storage disorder due to hepatic glycogen synthase deficiencyOpen Targets
0.04Suggestive
hyperproinsulinemiaOpen Targets
0.04Suggestive
hyperinsulinism due to INSR deficiencyOpen Targets
0.03Suggestive
prostate carcinomaOpen Targets
0.03Suggestive
prostate cancerOpen Targets
0.02Suggestive
Abnormality of the skeletal systemOpen Targets
0.02Suggestive
musculoskeletal system diseaseOpen Targets
0.02Suggestive
colorectal carcinomaOpen Targets
0.01Suggestive
gliomaOpen Targets
0.01Suggestive
neoplasmOpen Targets
0.01Suggestive
type 1 diabetes mellitusOpen Targets
0.01Suggestive
inflammatory bowel diseaseOpen Targets
0.01Suggestive
ulcerative colitisOpen Targets
0.01Suggestive
brain aneurysmOpen Targets
0.01Suggestive
cancerOpen Targets
0.00Suggestive
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Related Genes
RPL23Protein interaction100%MALSU1Protein interaction99%RPUSD2Protein interaction98%PUS7Protein interaction98%PUS7LProtein interaction98%MRPL11Protein interaction95%
Tissue Expression6 tissues
Liver
100%
Heart
75%
Bone Marrow
67%
Brain
44%
Ovary
40%
Lung
39%
Gene Interaction Network
Click a node to explore
MRM3RPL23MALSU1RPUSD2PUS7PUS7LMRPL11
PROTEIN STRUCTURE
Preparing viewer…
PDB7OI6 · 5.70 Å · EM
View on RCSB ↗
Constraintⓘ
LOEUFⓘ
1.16LoF Tolerant
pLIⓘ
0.00Tolerant
Observed/Expected LoF0.87 [0.66–1.16]
RankingsWhere MRM3 stands among ~20K protein-coding genes
  • #5,618of 20,598
    Most Researched85
  • #12,091of 17,882
    Most Constrained (LOEUF)1.16
Genes detectedMRM3
Sources retrieved10 papers
Response time—
📄 Sources
10▼
1
MRM2 and MRM3 are involved in biogenesis of the large subunit of the mitochondrial ribosome.
PMID: 25009282
Mol Biol Cell · 2014
1.00
2
A distinct assembly pathway of the human 39S late pre-mitoribosome.
PMID: 34315873
Nat Commun · 2021
0.90
3
Human Cytomegalovirus Infection Upregulates the Mitochondrial Transcription and Translation Machineries.
PMID: 27025248
mBio · 2016
0.80
4
A late-stage assembly checkpoint of the human mitochondrial ribosome large subunit.
PMID: 35177605
Nat Commun · 2022
0.70
5
A Novel Liquid Chromatography Tandem Mass Spectrometry Technique using Multi-Period-Multi-Experiment of MRM-EPI-MRM3 with Library Matching for Simultaneous Determination of Amphetamine Type Stimulants Related Drugs in Whole Blood, Urine and Dried Blood Stain (DBS)-Application to Forensic Toxicology Cases in Malaysia.
PMID: 31141150
J Anal Toxicol · 2019
0.60