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10 sources retrieved · Most recent: April 2026 · Index updated 14 days ago
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DXO
decapping exoribonuclease
Chromosome 6 · 6p21.33
NCBI Gene: 1797Ensembl: ENSG00000204348.11HGNC: HGNC:2992UniProt: A0A024RCW8
32PubMed Papers
0Diseases
0Drugs
0Pathogenic Variants
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
protein bindingNAD-cap decappingnucleusmRNA catabolic process
✦AI Summary

DXO is a multifunctional RNA processing enzyme that serves as a quality control mechanism for RNA cap structures in mammalian cells. Its primary function involves decapping activity against non-canonical RNA caps, specifically removing NAD+ caps from the 5'-end of RNAs through a 'deNADding' reaction 1. Unlike the canonical m7G cap which promotes RNA stability, NAD+ caps promote mRNA decay, and DXO efficiently removes these caps to regulate cellular RNA levels 1. DXO also exhibits decapping activity toward FAD-capped and dpCoA-capped RNAs 2. The enzyme functions as a pre-mRNA capping quality control system by degrading incompletely capped RNAs, showing activity against unmethylated 5'-end-capped RNA (cap0) while sparing properly modified 2'-O-methylated m7G caps (cap1) 3. DXO possesses both decapping and 5'-3' exoribonuclease activities, enabling it to completely degrade faulty RNA molecules 3. Located in the MHC class III region, DXO is part of an RNA metabolism and surveillance quartet critical for transcriptional and translational processes 4. The enzyme shows nuclear localization and may have clinical relevance, as it has been associated with immune-mediated diseases including celiac disease 5.

Sources cited
1
DXO removes NAD+ caps from RNAs through deNADding reaction and NAD+ caps promote mRNA decay
PMID: 28283058
2
DXO exhibits decapping activity toward FAD-capped and dpCoA-capped RNAs
PMID: 32374864
3
DXO degrades incompletely capped RNAs but spares 2'-O-methylated caps and has both decapping and exoribonuclease activities
PMID: 29601584
4
DXO is part of RNA metabolism and surveillance quartet in MHC class III region
PMID: 31480283
5
DXO is associated with celiac disease in immune-mediated disease analysis
PMID: 39009607
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Related Genes
DCP2Protein interaction100%XRN2Protein interaction99%RPRD1BProtein interaction93%DNASE1L2Protein interaction91%TJAP1Protein interaction91%EXOSC4Protein interaction86%
Tissue Expression

No tissue expression data available for this gene.

Gene Interaction Network
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DXODCP2XRN2RPRD1BDNASE1L2TJAP1EXOSC4
PROTEIN STRUCTURE
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AlphaFoldAI-predicted · UniProt O77932
View on AlphaFold ↗
Constraintⓘ
LOEUFⓘ
1.26LoF Tolerant
pLIⓘ
0.00Tolerant
Observed/Expected LoF0.98 [0.76–1.26]
RankingsWhere DXO stands among ~20K protein-coding genes
  • #11,504of 20,598
    Most Researched32
  • #13,330of 17,882
    Most Constrained (LOEUF)1.26
Genes detectedDXO
Sources retrieved10 papers
Response time—
📄 Sources
10▼
1
Large-scale whole-exome sequencing analyses identified protein-coding variants associated with immune-mediated diseases in 350,770 adults.
PMID: 39009607
Nat Commun · 2024
1.00
2
DXO/Rai1 enzymes remove 5'-end FAD and dephospho-CoA caps on RNAs.
PMID: 32374864
Nucleic Acids Res · 2020
0.90
3
5' End Nicotinamide Adenine Dinucleotide Cap in Human Cells Promotes RNA Decay through DXO-Mediated deNADding.
PMID: 28283058
Cell · 2017
0.80
4
An RNA Metabolism and Surveillance Quartet in the Major Histocompatibility Complex.
PMID: 31480283
Cells · 2019
0.70
5
2'-O-methylation of the mRNA cap protects RNAs from decapping and degradation by DXO.
PMID: 29601584
PLoS One · 2018
0.60