# PDGFRA

Source: https://onco.cc/targets/pdgfra/  
OnCo record `pdgfra` (Target). Data CC BY-NC 4.0, attribute "Data from OnCo (onco.cc)"; commercial use needs a licence.

## TL;DR

PDGFRA is a growth-factor receptor mutated in about 10% of GISTs, including the D842V mutation that resists imatinib but responds to avapritinib.

## Summary

PDGFRA activating mutations (exons 12, 14, 18) define KIT-wild-type GIST with gastric location and epithelioid morphology; D842V (~60% of PDGFRA-mutant GIST) is imatinib-resistant but responds to avapritinib (~90% response, 2020). FIP1L1-PDGFRA fusions cause hypereosinophilic syndrome/chronic eosinophilic leukaemia that is exquisitely imatinib-sensitive; PDGFRA amplification occurs in glioblastoma (~15%) without a proven therapy. Olaratumab (anti-PDGFRα) failed in sarcoma (ANNOUNCE).

## Fields

- Kind: Target
- Last checked: 2026-09-08
- Tags: gap-fill
- Symbol: PDGFRA
- Class: kinase
- Biology: Type III receptor tyrosine kinase (with KIT, CSF1R, FLT3); ligand PDGF-AA/BB dimerises the receptor, activating RAS/MAPK, PI3K and STAT pathways; D842V in the activation loop stabilises the active conformation.
- Where found: GIST (~10%, gastric, KIT-wild-type); Chronic eosinophilic leukaemia (FIP1L1-PDGFRA); Glioblastoma (amplification ~15%); Dermatofibrosarcoma protuberans (COL1A1-PDGFB, ligand-driven)

## Sources

- Wikipedia: https://en.wikipedia.org/wiki/PDGFRA
- Heinrich 2003 (Science): https://doi.org/10.1126/science.1079666

## Connected records

- targets: [CSF1R](https://onco.cc/targets/csf1r/), [KIT](https://onco.cc/targets/kit/)
- biomarkers: [PDGFRA exon 18 mutation (D842V)](https://onco.cc/biomarkers/pdgfra-exon-18-d842v/)
- cancers: [Chordoma](https://onco.cc/cancers/chordoma/), [Dermatofibrosarcoma protuberans](https://onco.cc/cancers/dermatofibrosarcoma-protuberans/), [Desmoplastic small round cell tumour](https://onco.cc/cancers/desmoplastic-small-round-cell-tumour/), [Gastrointestinal stromal tumour (GIST)](https://onco.cc/cancers/gist/), [Glioma & glioblastoma](https://onco.cc/cancers/glioblastoma/), [Inflammatory myofibroblastic tumour (IMT)](https://onco.cc/cancers/inflammatory-myofibroblastic-tumour/), [PDGFRA D842V-mutant GIST](https://onco.cc/cancers/gist-pdgfra-d842v/), [Sarcomas (soft tissue, bone, GIST)](https://onco.cc/cancers/sarcoma/)
- technologies: [Small-molecule kinase inhibitors](https://onco.cc/technologies/kinase-inhibitors/)
- drugs: [Avapritinib](https://onco.cc/drugs/avapritinib/), [Chiauranib](https://onco.cc/drugs/chiauranib/), [Imatinib](https://onco.cc/drugs/imatinib/), [Ripretinib](https://onco.cc/drugs/ripretinib/), [Sunitinib](https://onco.cc/drugs/sunitinib/)
- pathways: [Glioma (KEGG map)](https://onco.cc/pathways/glioma-signalling/), [PI3K / AKT / mTOR](https://onco.cc/pathways/pi3k-akt-mtor/), [RAS / RAF / MEK / ERK (MAPK)](https://onco.cc/pathways/ras-mapk/), [Receptor tyrosine kinase activation](https://onco.cc/pathways/rtk-activation/), [The angiogenic switch & tumour vessels](https://onco.cc/pathways/angiogenic-switch/)
- terms: [GIST risk stratification (mitotic count, size, site; Miettinen and modified NIH criteria)](https://onco.cc/terms/gist-risk-stratification/)

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