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25 sources retrieved · Most recent: April 2026 · Index updated 14 days ago
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MTREX
Mtr4 exosome RNA helicase
Chromosome 5 · 5q11.2
NCBI Gene: 23517Ensembl: ENSG00000039123.17HGNC: HGNC:18734UniProt: P42285
211PubMed Papers
20Diseases
0Drugs
0Pathogenic Variants
FUNCTIONAL ROLE
Hub Gene
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
nuclear exosome (RNase complex)exosome (RNase complex)maturation of 5.8S rRNArRNA processingmarfanoid habitus and intellectual disabilityhepatocellular carcinomagliomacancer
✦AI Summary

MTREX (MTR4) is an ATP-dependent RNA helicase that catalyzes unwinding of RNA duplexes with 3' single-stranded RNA extensions 12. It functions as a central subunit of multiple RNA surveillance complexes including TRAMP-like, NEXT (nuclear exosome targeting), and PAXT (poly(A) tail exosome targeting) 12. MTREX directs non-coding and aberrant transcripts for exosomal degradation and participates in 3'-processing of pre-rRNA to mature 5.8S rRNA 3. Beyond its canonical exosome functions, MTREX interacts with splicing factors and transcription elongation complexes, regulating intron retention and gene expression more broadly than previously recognized 4. MTREX regulates RNA exosome subcellular localization within the nucleolus, with MTR4 depletion causing exosome translocation to the nucleoplasm 5. Functionally, MTREX participates in DNA damage response and nuclear retention of viral RNA. Clinically, MTREX expression correlates with cognitive markers in schizophrenia patients exposed to cannabis 6. MTREX also plays roles in HIV-1 latency maintenance through RNA surveillance mechanisms 78 and regulates nuclear paraspeckle-associated lncRNA degradation 9.

Sources cited
1
MTREX catalyzes ATP-dependent unwinding of RNA duplexes and is central to NEXT complex
PMID: 27871484
2
MTREX unwinds RNA duplexes and DNA:RNA heteroduplexes as part of NEXT complex
PMID: 29844170
3
MTREX associates with exosome and is involved in 3' end processing of 5.8S rRNA
PMID: 17412707
4
MTREX interacts with splicing factors and transcription elongation complexes, broadly impacting gene expression
PMID: 41097056
5
MTR4 regulates RNA exosome localization within the nucleolus
PMID: 40651665
6
MTREX expression correlates with cognitive markers and clinical signs in schizophrenia patients using cannabis
PMID: 33668817
7
MTR4 regulates nuclear retention of unspliced HIV-1 RNA in latency
PMID: 40021667
8
MTR4 is part of nuclear RNA surveillance network that silences HIV-1 transcription
PMID: 29554134
9
MTREX associates with hnRNPH1 to mediate degradation of NEAT1v2 lncRNA
PMID: 34470577
Disease Associationsⓘ20
marfanoid habitus and intellectual disabilityOpen Targets
0.12Weak
hepatocellular carcinomaOpen Targets
0.08Suggestive
gliomaOpen Targets
0.06Suggestive
cancerOpen Targets
0.04Suggestive
nasopharyngeal carcinomaOpen Targets
0.04Suggestive
neoplasmOpen Targets
0.03Suggestive
gastrointestinal diseaseOpen Targets
0.02Suggestive
intestinal diseaseOpen Targets
0.02Suggestive
ovarian cancerOpen Targets
0.01Suggestive
viral diseaseOpen Targets
0.01Suggestive
dysplasia of cervixOpen Targets
0.01Suggestive
human papilloma virus infectionOpen Targets
0.01Suggestive
leukemiaOpen Targets
0.01Suggestive
male infertilityOpen Targets
0.01Suggestive
HIV-1 infectionOpen Targets
0.01Suggestive
prostate adenocarcinomaOpen Targets
0.01Suggestive
infectionOpen Targets
0.00Suggestive
multiple myelomaOpen Targets
0.00Suggestive
Myocardial IschemiaOpen Targets
0.00Suggestive
posterior cortical atrophyOpen Targets
0.00Suggestive
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Related Genes
EXOSC9Protein interaction100%RBM7Protein interaction100%C1DProtein interaction100%EXOSC8Protein interaction100%EXOSC7Protein interaction100%EXOSC2Protein interaction100%
Tissue Expression6 tissues
Heart
100%
Brain
93%
Ovary
70%
Lung
64%
Bone Marrow
63%
Liver
57%
Gene Interaction Network
Click a node to explore
MTREXEXOSC9RBM7C1DEXOSC8EXOSC7EXOSC2
PROTEIN STRUCTURE
Preparing viewer…
PDB6C90 · 2.20 Å · X-ray
View on RCSB ↗
Constraintⓘ
LOEUFⓘ
0.65LoF Tolerant
pLIⓘ
0.02Tolerant
Observed/Expected LoF0.46 [0.33–0.65]
RankingsWhere MTREX stands among ~20K protein-coding genes
  • #1,978of 20,598
    Most Researched211 · top 10%
  • #4,663of 17,882
    Most Constrained (LOEUF)0.65
Genes detectedMTREX
Sources retrieved25 papers
Response time—
📄 Sources
25▼
1
NRDE2 Interacts with an Early Transcription Elongation Complex and Widely Impacts Gene Expression.
PMID: 41097056
Int J Mol Sci · 2025
1.00
2
Nuclear retention of unspliced HIV-1 RNA as a reversible post-transcriptional block in latency.
PMID: 40021667
Nat Commun · 2025
0.90
3
hnRNPH1-MTR4 complex-mediated regulation of
PMID: 34470577
RNA Biol · 2021
0.80
4
MTR4 adaptor PICT1 functions in two distinct steps during pre-rRNA processing.
PMID: 36403484
Biochem Biophys Res Commun · 2022
0.76
5
Mtr4 RNA helicase structures and interactions.
PMID: 33857361
Biol Chem · 2021
0.70