HomeAboutRankingsData Sources
© 2026 GeneE
🧬
GeneE
10 sources retrieved · Most recent: April 2026 · Index updated 14 days ago
ⓘGeneE is for informational purposes only. It is not a substitute for professional medical advice, diagnosis, or treatment.
MON2
MON2 regulator of endosome-to-Golgi trafficking
Chromosome 12 · 12q14.1
NCBI Gene: 23041Ensembl: ENSG00000061987.16HGNC: HGNC:29177UniProt: A0A286YFF8
83PubMed Papers
20Diseases
0Drugs
0Pathogenic Variants
FUNCTIONAL ROLE
Transporter
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
protein bindingextracellular exosomeearly endosome membraneGolgi apparatusneurodegenerative diseaselysosomal storage diseasestricturebursitis
✦AI Summary

MON2 is a regulator of endosome-to-Golgi membrane trafficking that plays a critical role in retrograde transport and cargo sorting. Mechanistically, MON2 functions as part of a conserved complex with DOPEY proteins and ATP9A to mediate SNX3-retromer-dependent endosomal sorting 1. MON2 binds phosphatidic acid and localizes to the Golgi, endolysosomes, and endoplasmic reticulum exit sites, where it recruits kinesin-1 motor proteins for microtubule-dependent transport 2. The MON2-DOPEY complex drives separation of recycling endosomes from early endosomes and is specifically required for Wntless transport to the Golgi, preventing its lysosomal degradation 3. Beyond membrane trafficking, MON2 has emerged as a novel immune-oncology target with therapeutic potential, as it was identified in CRISPR screening studies as a regulator of tumor-immune interactions affecting response to immunotherapy 4. Additionally, rare coding variants in MON2 have been associated with neuroticism in large-scale exome sequencing studies 5. MON2 demonstrates clinical significance in cardiovascular disease, where abnormal monocyte subset Mon2 (CD14++CD16+CCR2+) characteristics independently predict poor outcomes following myocardial infarction and correlate with microvascular endothelial dysfunction 67. Novel MON2 gene fusions have also been identified in diagnostically challenging head and neck carcinomas 8.

Sources cited
1
MON2 associates with DOPEY2 and ATP9A in an evolutionary conserved complex required for SNX3-retromer-mediated Wntless sorting and endosome-to-Golgi transport
PMID: 30213940
2
MON2 binds phosphatidic acid, localizes to Golgi and endolysosomes, and recruits kinesin-1 motor protein for microtubule-dependent bidirectional transport
PMID: 31324769
3
MON2 drives separation of recycling endosomes from early endosomes and is required for Wntless retrograde transport and intracellular cargo recycling
PMID: 32404555
4
MON2 identified as a novel immune-oncology target with therapeutic potential through CRISPR screening integrative analysis
PMID: 34980132
5
MON2 rare coding variants associated with neuroticism in large-scale exome sequencing of UK Biobank individuals
PMID: 39511343
6
Mon2 monocyte subset counts independently predict major adverse cardiovascular events and heart failure outcomes in ST-elevation myocardial infarction
PMID: 30644612
7
Monocyte-platelet aggregates with Mon2 subset independently predict diffuse coronary artery disease and correlate negatively with endothelium-dependent microvascular vasodilation
PMID: 29423944
8
MON2::STAT6 gene fusion identified in head and neck carcinoma as a novel chromosomal rearrangement
PMID: 39628352
Disease Associationsⓘ20
neurodegenerative diseaseOpen Targets
0.56Moderate
lysosomal storage diseaseOpen Targets
0.37Weak
strictureOpen Targets
0.31Weak
bursitisOpen Targets
0.30Weak
liver diseaseOpen Targets
0.29Weak
paralytic strabismusOpen Targets
0.28Weak
type 2 diabetes mellitusOpen Targets
0.26Weak
response to antihypertensive drugOpen Targets
0.15Weak
hypertensionOpen Targets
0.12Weak
alcohol drinkingOpen Targets
0.11Weak
smoking initiationOpen Targets
0.10Weak
Griscelli diseaseOpen Targets
0.06Suggestive
aortic aneurysmOpen Targets
0.06Suggestive
Griscelli disease type 3Open Targets
0.05Suggestive
Griscelli syndrome type 3Open Targets
0.05Suggestive
Abnormal nasolacrimal system morphologyOpen Targets
0.05Suggestive
injuryOpen Targets
0.05Suggestive
uncombable hair syndromeOpen Targets
0.04Suggestive
AscitesOpen Targets
0.04Suggestive
oculocutaneous albinism type 3Open Targets
0.04Suggestive
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Related Genes
VPS26BProtein interaction99%MIA3Protein interaction77%VPS26AProtein interaction76%FBXO38Protein interaction75%ATG2BProtein interaction73%ARL1Protein interaction71%
Tissue Expression6 tissues
Liver
100%
Ovary
97%
Bone Marrow
87%
Brain
69%
Lung
69%
Heart
54%
Gene Interaction Network
Click a node to explore
MON2VPS26BMIA3VPS26AFBXO38ATG2BARL1
PROTEIN STRUCTURE
Preparing viewer…
AlphaFoldAI-predicted · UniProt Q7Z3U7
View on AlphaFold ↗
Constraintⓘ
LOEUFⓘ
0.43Moderately Constrained
pLIⓘ
1.00Intolerant
Observed/Expected LoF0.34 [0.27–0.43]
RankingsWhere MON2 stands among ~20K protein-coding genes
  • #5,739of 20,598
    Most Researched83
  • #2,274of 17,882
    Most Constrained (LOEUF)0.43 · top quartile
Genes detectedMON2
Sources retrieved10 papers
Response time—
📄 Sources
10▼
1
Targeted RNA sequencing in diagnostically challenging head and neck carcinomas identifies novel MON2::STAT6, NFATC2::NUTM2B, POC5::RAF1, and NSD3::NCOA2 gene fusions.
PMID: 39628352
Histopathology · 2025
1.00
2
Dopey1-Mon2 complex binds to dual-lipids and recruits kinesin-1 for membrane trafficking.
PMID: 31324769
Nat Commun · 2019
0.90
3
Integrative analysis of CRISPR screening data uncovers new opportunities for optimizing cancer immunotherapy.
PMID: 34980132
Mol Cancer · 2022
0.80
4
Mon2 predicts poor outcome in ST-elevation myocardial infarction.
PMID: 30644612
J Intern Med · 2019
0.70
5
Large-scale exome sequencing identified 18 novel genes for neuroticism in 394,005 UK-based individuals.
PMID: 39511343
Nat Hum Behav · 2025
0.60