Atypical teratoid rhabdoid tumour (AT/RT)
A SMARCB1-deficient brain tumour of infants and young children: EZH2 inhibition and intensive chemotherapy.
Each dot is one target. Higher means stronger genetic evidence in people (inherited variants or tumour mutations, whichever is stronger). Further right means more evidence from drugs already in trials or approved.
Scores run from 0 to 1 and are Open Targets' summary of how much evidence exists, not the chance a drug will work. The dividing lines sit at 0.5. Hover a dot for its numbers, click it for the target's page. Source: Open Targets Platform, fetched live.
The 4 targets in the top-left of the chart, strongest genetic evidence first. Click one for its drugs, tractability and other cancers.
All 50 targets, by type of evidence
Ranked by overall score for atypical teratoid rhabdoid tumor on the Open Targets Platform. Darker cells mean stronger evidence of that type.
Peds RMTLmarks the 3 of these targets on the FDA's Pediatric Relevant Molecular Target List: a drug against one of them can be required to be studied in children under the RACE for Children Act.
| Target | Overall | Germline genetics | Somatic | genetic literature | Literature | Animal models | Clinical | RNA expr. |
|---|---|---|---|---|---|---|---|---|
| SMARCA4 SWI/SNF related BAF chromatin remodeling complex subunit ATPase 4 | 0.80 | 0.95 | 0.46 | |||||
| SMARCB1 SWI/SNF related BAF chromatin remodeling complex subunit B1 | 0.48 | 0.24 | 0.61 | |||||
| TP53Peds RMTL tumor protein p53 | 0.38 | 0.55 | 0.30 | |||||
| TUBB4A tubulin beta 4A class IVa | 0.35 | – | – | |||||
| TUBA4A tubulin alpha 4a | 0.34 | – | – | |||||
| TUBA3C tubulin alpha 3c | 0.33 | – | – | |||||
| TUBA1C tubulin alpha 1c | 0.33 | – | – | |||||
| TUBB6 tubulin beta 6 class V | 0.33 | – | – | |||||
| TUBB3 tubulin beta 3 class III | 0.33 | – | – | |||||
| TUBA1A tubulin alpha 1a | 0.33 | – | – | |||||
| TUBB8 tubulin beta 8 class VIII | 0.33 | – | – | |||||
| TUBB2A tubulin beta 2A class IIa | 0.33 | – | – | |||||
| TUBB tubulin beta class I | 0.33 | – | – | |||||
| TUBB4B tubulin beta 4B class IVb | 0.33 | – | – | |||||
| TUBB2B tubulin beta 2B class IIb | 0.33 | – | – | |||||
| TUBA3E tubulin alpha 3e | 0.33 | – | – | |||||
| TUBA1B tubulin alpha 1b | 0.33 | – | – | |||||
| TUBB1 tubulin beta 1 class VI | 0.33 | – | – | |||||
| ATRXPeds RMTL ATRX chromatin remodeler | 0.33 | 0.55 | – | |||||
| TOP2A DNA topoisomerase II alpha | 0.31 | – | – | |||||
| DHFR dihydrofolate reductase | 0.29 | – | – | |||||
| TOP2B DNA topoisomerase II beta | 0.29 | – | – | |||||
| BRAFPeds RMTL B-Raf proto-oncogene, serine/threonine kinase | 0.28 | – | 0.46 | |||||
| CSF3R colony stimulating factor 3 receptor | 0.28 | – | – | |||||
| NF2 NF2, moesin-ezrin-radixin like (MERLIN) tumor suppressor | 0.19 | – | 0.30 | |||||
| NCOR2 nuclear receptor corepressor 2 | 0.18 | – | 0.30 | |||||
| MSH2 mutS homolog 2 | 0.18 | – | 0.30 | |||||
| GATA2 GATA binding protein 2 | 0.18 | – | 0.30 | |||||
| ERBB4 erb-b2 receptor tyrosine kinase 4 | 0.18 | – | 0.30 | |||||
| FANCD2 FA complementation group D2 | 0.18 | – | 0.30 | |||||
| NRAS NRAS proto-oncogene, GTPase | 0.18 | – | 0.30 | |||||
| MDM4 MDM4 regulator of p53 | 0.18 | – | 0.30 | |||||
| RICTOR RPTOR independent companion of MTOR complex 2 | 0.18 | – | 0.30 | |||||
| KRAS KRas proto-oncogene, GTPase | 0.18 | – | 0.30 | |||||
| MAF MAF bZIP transcription factor | 0.18 | – | 0.30 | |||||
| BCL11B BCL11 transcription factor B | 0.18 | – | 0.30 | |||||
| SPEN spen family transcriptional repressor | 0.18 | – | 0.30 | |||||
| ZFHX3 zinc finger homeobox 3 | 0.18 | – | 0.30 | |||||
| BRCA2 BRCA2 DNA repair associated | 0.12 | 0.19 | – | |||||
| MYO5A myosin VA | 0.11 | – | 0.15 | |||||
| POLE2 DNA polymerase epsilon 2, accessory subunit | 0.11 | – | – | |||||
| POLA1 DNA polymerase alpha 1, catalytic subunit | 0.10 | – | – | |||||
| POLD4 DNA polymerase delta 4, accessory subunit | 0.10 | – | – | |||||
| POLD1 DNA polymerase delta 1, catalytic subunit | 0.10 | – | – | |||||
| POLE DNA polymerase epsilon, catalytic subunit | 0.10 | – | – | |||||
| POLA2 DNA polymerase alpha 2, accessory subunit | 0.10 | – | – | |||||
| POLD3 DNA polymerase delta 3, accessory subunit | 0.10 | – | – | |||||
| PRIM2 DNA primase subunit 2 | 0.10 | – | – | |||||
| POLD2 DNA polymerase delta 2, accessory subunit | 0.10 | – | – | |||||
| POLE3 DNA polymerase epsilon 3, accessory subunit | 0.10 | – | – |
1 biomarker in Atypical teratoid rhabdoid tumour (AT/RT) has validated or clinical evidence in CIViC
Accepted evidence from CIViC, the open (CC0) expert-curated knowledgebase, searched by the disease name “Atypical Teratoid Rhabdoid Tumor”. Level A is validated (for example in guidelines or an approval), level B comes from clinical trials or studies. 1 accepted items exist; the first 1 were read. This is the published evidence, not treatment advice.
- SMARCB1 Loss level B