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10 sources retrieved · Most recent: April 2026 · Index updated 15 days ago
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CYSLTR2
cysteinyl leukotriene receptor 2
Chromosome 13 · 13q14.2
NCBI Gene: 57105Ensembl: ENSG00000152207.9HGNC: HGNC:18274UniProt: A4ZKH2
77PubMed Papers
20Diseases
0Drugs
0Pathogenic Variants
FUNCTIONAL ROLE
Receptor
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
protein bindinggalanin receptor activitycysteinyl leukotriene receptor activityplasma membraneventricular fibrillationUveal Melanomapreeclampsiaovarian neoplasm
✦AI Summary

CYSLTR2 encodes cysteinyl leukotriene receptor 2, a G-protein-coupled receptor that responds to cysteinyl leukotrienes (LTC4, LTD4, LTE4) with LTC4 showing the highest affinity 1. The receptor activates phosphatidylinositol-calcium second messenger systems and mediates immune and inflammatory responses [UniProt]. Beyond classical leukotriene signaling, CYSLTR2 functions as an endogenous receptor for long-chain ceramides (C16:0 and C20:0), which bind to a channel-like pocket on the receptor and activate Gq-mediated inflammasome signaling in endothelial cells and macrophages 2. This ceramide-sensing function contributes to atherosclerosis progression and chr13 kidney disease-associated vascular disease 2. In allergic diseases, CYSLTR2 variants influence individual response to leukotriene-modifying asthma medications 3. CYSLTR2 is highly expressed in dorsal root ganglia neurons and mediates LTC4-induced itch in allergic skin inflammation and atopic dermatitis models, providing a therapeutic target for inflammatory itch 1. Clinically, CYSLTR2 mutations represent rare but significant oncogenic drivers in melanoma. Activating CYSLTR2 mutations (particularly p.L129Q) initiate uveal melanoma development through G-protein signaling activation, constituting an alternative to more common GNAQ/GNA11 mutations 45. CYSLTR2 mutations also occur in malignant blue melanoma, a cutaneous variant with poor prognosis 6. These findings establish CYSLTR2 as a dual-function receptor linking inflammatory signaling to oncogenic transformation.

Sources cited
1
CYSLTR2 functions as endogenous receptor for long-chain ceramides C16:0 and C20:0, activates inflammasome signaling, and contributes to atherosclerosis and chronic kidney disease progression
PMID: 40049228
2
CYSLTR2 is expressed in dorsal root ganglia neurons and mediates LTC4-induced itch in allergic skin inflammation and atopic dermatitis models
PMID: 33753496
3
CYSLTR2 variants contribute to variability in response to leukotriene modifier drugs in asthma patients
PMID: 19077707
4
CYSLTR2 p.L129Q activating mutation initiates uveal melanoma development and wild-type expression correlates with tumor inflammation
PMID: 33588787
5
CYSLTR2 mutations activate G protein-coupled receptor signaling in uveal melanoma as rare alternative to GNAQ/GNA11 mutations
PMID: 29738114
6
CYSLTR2 mutations occur in malignant blue melanoma as rare genetic drivers
PMID: 39260463
7
Cysteinyl leukotrienes including LTC4 play key roles in asthma pathophysiology
PMID: 11676142
Disease Associationsⓘ20
ventricular fibrillationOpen Targets
0.31Weak
Uveal MelanomaOpen Targets
0.29Weak
preeclampsiaOpen Targets
0.28Weak
ovarian neoplasmOpen Targets
0.26Weak
melanomaOpen Targets
0.20Weak
clavicle fractureOpen Targets
0.19Weak
shoulder fractureOpen Targets
0.19Weak
papillary thyroid carcinomaOpen Targets
0.19Weak
hepatocellular carcinomaOpen Targets
0.19Weak
esophageal squamous cell carcinomaOpen Targets
0.19Weak
colorectal adenocarcinomaOpen Targets
0.19Weak
acute myeloid leukemiaOpen Targets
0.19Weak
lung adenocarcinomaOpen Targets
0.19Weak
multiple myelomaOpen Targets
0.19Weak
cutaneous melanomaOpen Targets
0.19Weak
brain glioblastomaOpen Targets
0.18Weak
carcinoma of liver and intrahepatic biliary tractOpen Targets
0.18Weak
Endometrial Endometrioid AdenocarcinomaOpen Targets
0.18Weak
gastric adenocarcinomaOpen Targets
0.18Weak
oral squamous cell carcinomaOpen Targets
0.18Weak
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Related Genes
GNAQProtein interaction97%GNA11Protein interaction89%GNA15Protein interaction89%PLCB4Protein interaction89%TBXA2RProtein interaction89%GNA14Protein interaction89%
Tissue Expression6 tissues
Heart
100%
Bone Marrow
23%
Brain
7%
Ovary
6%
Lung
3%
Liver
2%
Gene Interaction Network
Click a node to explore
CYSLTR2GNAQGNA11GNA15PLCB4TBXA2RGNA14
PROTEIN STRUCTURE
Preparing viewer…
PDB9JH5 · 2.76 Å · EM
View on RCSB ↗
Constraintⓘ
LOEUFⓘ
1.62LoF Tolerant
pLIⓘ
0.45Tolerant
Observed/Expected LoF0.00 [0.00–1.62]
RankingsWhere CYSLTR2 stands among ~20K protein-coding genes
  • #6,147of 20,598
    Most Researched77
  • #15,739of 17,882
    Most Constrained (LOEUF)1.62
Genes detectedCYSLTR2
Sources retrieved10 papers
Response time—
📄 Sources
10▼
1
Sensing ceramides by CYSLTR2 and P2RY6 to aggravate atherosclerosis.
PMID: 40049228
Nature · 2025
1.00
2
Malignant blue melanoma.
PMID: 39260463
Clin Dermatol · 2025
0.90
3
Pharmacogenetics of asthma.
PMID: 19077707
Curr Opin Pulm Med · 2009
0.80
4
The CysLT
PMID: 33753496
Proc Natl Acad Sci U S A · 2021
0.70
5
Recent Advances in Molecular and Genetic Research on Uveal Melanoma.
PMID: 38920653
Cells · 2024
0.60