Missing content? – Request curation!
Request curation for specific Genes, Variants, or PubMed publications.
Have questions, comments, or suggestions? - Let us know!
Email us at : ckbsupport@genomenon.com
Gene | ATM |
Variant | R3008H |
Impact List | missense |
Protein Effect | loss of function |
Gene Variant Descriptions | ATM R3008H does not lie within any known functional domains of the Atm protein (UniProt.org). R3008H results in kinase activity, and recruitment to DNA damage similar to wild-type Atm protein, but decreased Atm activation, altered irradiation-induced cell cycle checkpoint, partial loss of tumor suppressor function (PMID: 33239428), and failure to induce Tp53 target gene expression after DNA damage in patient-derived cells (PMID: 23585524). |
Associated Drug Resistance | |
Category Variants Paths |
ATM mutant ATM inact mut ATM R3008H ATM mutant ATM R3008X ATM R3008H |
Transcript | NM_000051.4 |
gDNA | chr11:g.108365360G>A |
cDNA | c.9023G>A |
Protein | p.R3008H |
Source Database | RefSeq |
Genome Build | GRCh38/hg38 |
Transcript | gDNA | cDNA | Protein | Source Database | Genome Build |
---|---|---|---|---|---|
XM_005271562.6 | chr11:g.108365360G>A | c.9023G>A | p.R3008H | RefSeq | GRCh38/hg38 |
NM_000051.4 | chr11:g.108365360G>A | c.9023G>A | p.R3008H | RefSeq | GRCh38/hg38 |
XM_005271562 | chr11:g.108365360G>A | c.9023G>A | p.R3008H | RefSeq | GRCh38/hg38 |
XM_006718843.4 | chr11:g.108365360G>A | c.9023G>A | p.R3008H | RefSeq | GRCh38/hg38 |
XM_006718843 | chr11:g.108365360G>A | c.9023G>A | p.R3008H | RefSeq | GRCh38/hg38 |
XM_017017790 | chr11:g.108365360G>A | c.9023G>A | p.R3008H | RefSeq | GRCh38/hg38 |
XM_017017790.3 | chr11:g.108365360G>A | c.9023G>A | p.R3008H | RefSeq | GRCh38/hg38 |
NM_000051 | chr11:g.108365360G>A | c.9023G>A | p.R3008H | RefSeq | GRCh38/hg38 |
XM_011542840.4 | chr11:g.108365360G>A | c.9023G>A | p.R3008H | RefSeq | GRCh38/hg38 |
XM_017017789 | chr11:g.108365360G>A | c.9023G>A | p.R3008H | RefSeq | GRCh38/hg38 |
XM_005271561 | chr11:g.108365360G>A | c.9023G>A | p.R3008H | RefSeq | GRCh38/hg38 |
NM_001351834.2 | chr11:g.108365360G>A | c.9023G>A | p.R3008H | RefSeq | GRCh38/hg38 |
NM_001351834.1 | chr11:g.108365360G>A | c.9023G>A | p.R3008H | RefSeq | GRCh38/hg38 |
XM_017017790.2 | chr11:g.108365360G>A | c.9023G>A | p.R3008H | RefSeq | GRCh38/hg38 |
XM_005271562.5 | chr11:g.108365360G>A | c.9023G>A | p.R3008H | RefSeq | GRCh38/hg38 |
XM_047426976.1 | chr11:g.108365360G>A | c.9023G>A | p.R3008H | RefSeq | GRCh38/hg38 |
NM_000051.3 | chr11:g.108365360G>A | c.9023G>A | p.R3008H | RefSeq | GRCh38/hg38 |
XM_011542840.3 | chr11:g.108365360G>A | c.9023G>A | p.R3008H | RefSeq | GRCh38/hg38 |
XM_017017789.2 | chr11:g.108365360G>A | c.9023G>A | p.R3008H | RefSeq | GRCh38/hg38 |
XM_011542840 | chr11:g.108365360G>A | c.9023G>A | p.R3008H | RefSeq | GRCh38/hg38 |
XM_006718843.5 | chr11:g.108365360G>A | c.9023G>A | p.R3008H | RefSeq | GRCh38/hg38 |
XM_047426975.1 | chr11:g.108365360G>A | c.9023G>A | p.R3008H | RefSeq | GRCh38/hg38 |
Molecular Profile | Indication/Tumor Type | Response Type | Therapy Name | Approval Status | Evidence Type | Efficacy Evidence | References |
---|---|---|---|---|---|---|---|
ATM R3008H | prostate cancer | predicted - sensitive | RP-3500 | Case Reports/Case Series | Actionable | In a Phase I/II trial (TRESR), RP-3500 treatment resulted in a 29% tumor reduction in a castration-resistant prostate cancer patient harboring ATM R3008H, who remained on treatment for more than 61 weeks (PMID: 37277454; NCT04497116). | 37277454 |
ATM R3008H | esophagus small cell carcinoma | predicted - sensitive | Etoposide + Olaparib | Case Reports/Case Series | Actionable | In a clinical case study, the combination of Lynparza (olaparib) and Vepesid (etoposide) resulted in a partial response and 5.9 months of progression-free survival in a patient with small cell esophageal carcinoma harboring ATM R3008H (PMID: 35480123). | 35480123 |