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26 sources retrieved · Most recent: April 2026 · Index updated 14 days ago
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ROS1
ROS proto-oncogene 1, receptor tyrosine kinase
Chromosome 6 · 6q22.1
NCBI Gene: 6098Ensembl: ENSG00000047936.11HGNC: HGNC:10261UniProt: P08922
218PubMed Papers
20Diseases
4Drugs
5Pathogenic Variants
FUNCTIONAL ROLE
KinaseOncogeneReceptor
RESEARCH IMPACT
Trending
CLINICAL
FDA Approved Target
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
regulation of cell growthregulation of TOR signalingprotein phosphorylationprotein bindingnon-small cell lung carcinomaneoplasmlung adenocarcinomacancer
✦AI Summary

ROS1 is a receptor tyrosine kinase that mediates cellular differentiation and proliferation through multiple signaling pathways 1. In normal physiology, ROS1 has an unknown physiological role in humans, though it plays a documented role in epithelial cell differentiation and regionalization of the proximal epididymal epithelium. ROS1 activates downstream signaling cascades including PI3 kinase-mTOR, MAPK/ERK, and STAT3 pathways through phosphorylation of substrates such as PTPN11, VAV3, and AKT1. Clinically, ROS1 fusion proteins drive oncogenic transformation in approximately 1-2% of non-small cell lung carcinoma and other malignancies including cholangiocarcinoma, gastric carcinoma, and inflammatory myofibroblastic tumors 23. These fusions produce chimeric oncoproteins with constitutive kinase activity that regulate cellular proliferation and are implicated in carcinogenesis 2. Multiple FDA-approved tyrosine kinase inhibitors—including crizotinib, entrectinib, repotrectinib, and the recently approved taletrectinib—target ROS1 fusion-positive cancers and have transformed clinical outcomes 45. However, acquired resistance invariably develops, often through solvent-front kinase domain mutations (e.g., ROS1 G2032R), necessitating sequential next-generation TKI therapy with improved CNS penetration and resistance mutation coverage 67.

Sources cited
1
ROS1 encodes a receptor tyrosine kinase with unknown physiological role in humans; somatic fusions drive diverse cancers; TKI resistance mechanisms are relevant
PMID: 32760015
2
ROS1 undergoes gene rearrangements in ~1-2% of NSCLC and other tumors; ROS1 fusion causes constitutive activation regulating cellular proliferation and carcinogenesis
PMID: 28903995
3
ROS1 rearrangement occurs in inflammatory myofibroblastic tumors and can be detected as TFG-ROS1 fusion transcript
PMID: 26647767
4
ROS1 fusions comprise 1-2% of NSCLC; FDA-approved first-line TKIs include crizotinib, entrectinib, and repotrectinib; TKI resistance invariably develops
PMID: 39177972
5
ROS1 solvent-front mutations (G2032R, D2033N) represent major acquired resistance mechanisms; repotrectinib overcomes these mutations
PMID: 30093503
6
Taletrectinib is a next-generation ROS1 inhibitor recently approved for advanced ROS1-positive NSCLC in China, USA, and Japan
PMID: 41222831
7
ROS1 encodes a receptor tyrosine kinase structurally homologous to ALK and TRK; repotrectinib demonstrates superior efficacy and CNS penetration compared to earlier-generation ROS1 TKIs; median PFS in TKI-naïve patients was 35.7 months
PMID: 39402859
Disease Associationsⓘ20
non-small cell lung carcinomaOpen Targets
0.71Strong
neoplasmOpen Targets
0.63Moderate
lung adenocarcinomaOpen Targets
0.59Moderate
cancerOpen Targets
0.47Moderate
lung carcinomaOpen Targets
0.46Moderate
melanomaOpen Targets
0.39Weak
squamous cell lung carcinomaOpen Targets
0.37Weak
skin basal cell carcinomaOpen Targets
0.37Weak
spitz nevusOpen Targets
0.37Weak
melanocytic neoplasmOpen Targets
0.37Weak
cutaneous melanomaOpen Targets
0.37Weak
Meningothelial MeningiomaOpen Targets
0.37Weak
bile duct carcinomaOpen Targets
0.37Weak
lymphoid neoplasmOpen Targets
0.37Weak
carcinoma of liver and intrahepatic biliary tractOpen Targets
0.37Weak
hemangioblastomaOpen Targets
0.37Weak
Merkel cell skin cancerOpen Targets
0.37Weak
Ovarian Endometrioid Adenocarcinoma with Squamous DifferentiationOpen Targets
0.37Weak
superficial spreading melanomaOpen Targets
0.37Weak
Transitional MeningiomaOpen Targets
0.37Weak
Pathogenic Variants5
NM_001378902.1(ROS1):c.5588dup (p.Ile1864fs)Likely pathogenic
Male infertility with azoospermia or oligozoospermia due to single gene mutation
★☆☆☆2023→ Residue 1864
NM_001378902.1(ROS1):c.3611T>A (p.Leu1204Ter)Likely pathogenic
Male infertility with azoospermia or oligozoospermia due to single gene mutation
★☆☆☆2023→ Residue 1204
NM_001378902.1(ROS1):c.3388G>A (p.Gly1130Arg)Pathogenic
Lung adenocarcinoma
★☆☆☆2022→ Residue 1130
NM_001378902.1(ROS1):c.4583del (p.Asn1528fs)Pathogenic
Short stature
☆☆☆☆2001→ Residue 1528
NM_001378902.1(ROS1):c.6076G>A (p.Gly2026Arg)Pathogenic
Lung adenocarcinoma
☆☆☆☆→ Residue 2026
View on ClinVar ↗
Drug Targets4
CRIZOTINIBApproved
EML4-ALK inhibitor
non-small cell lung carcinoma
ENTRECTINIBApproved
ALK tyrosine kinase receptor inhibitor
non-small cell lung carcinoma
REPOTRECTINIBApproved
NT-3 growth factor receptor inhibitor
non-small cell lung carcinoma
TALETRECTINIBPhase III
Neurotrophic tyrosine kinase receptor inhibitor
non-small cell lung carcinoma
Related Genes
PIK3CAProtein interaction100%EML4Protein interaction98%NRASProtein interaction94%OR13G1Protein interaction92%TAS2R50Protein interaction91%TPM3Protein interaction85%
Tissue Expression6 tissues
Lung
100%
Brain
2%
Liver
0%
Heart
0%
Bone Marrow
0%
Ovary
0%
Gene Interaction Network
Click a node to explore
ROS1PIK3CAEML4NRASOR13G1TAS2R50TPM3
PROTEIN STRUCTURE
Preparing viewer…
PDB7Z5X · 2.04 Å · X-ray
View on RCSB ↗
Constraintⓘ
LOEUFⓘ
0.99LoF Tolerant
pLIⓘ
0.00Tolerant
Observed/Expected LoF0.88 [0.78–0.99]
RankingsWhere ROS1 stands among ~20K protein-coding genes
  • #1,899of 20,598
    Most Researched218 · top 10%
  • #463of 1,025
    FDA-Approved Drug Targets3
  • #3,481of 5,498
    Most Pathogenic Variants5
  • #9,546of 17,882
    Most Constrained (LOEUF)0.99
Genes detectedROS1
Sources retrieved26 papers
Response time—
📄 Sources
26▼
1
Advances and future directions in ROS1 fusion-positive lung cancer.
PMID: 39177972
Oncologist · 2024
1.00
2
ROS1-dependent cancers - biology, diagnostics and therapeutics.
PMID: 32760015
Nat Rev Clin Oncol · 2021
0.90
3
ROS1 [corrected].
PMID: 28903995
J Clin Pathol · 2017
0.80
4
[ROS1-Translocations in Non-Small Cell Lung Cancer].
PMID: 26258422
Pneumologie · 2015
0.80
5
Repotrectinib (TPX-0005) Is a Next-Generation ROS1/TRK/ALK Inhibitor That Potently Inhibits ROS1/TRK/ALK Solvent- Front Mutations.
PMID: 30093503
Cancer Discov · 2018
0.70