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Gene | TP53 |
Variant | R248Q |
Impact List | missense |
Protein Effect | loss of function |
Gene Variant Descriptions | TP53 R248Q is a hotspot mutation that lies within the DNA-binding domain of the Tp53 protein (PMID: 22713868). R248Q results in increased proliferation, migration, invasion, and protein stability and altered subcellular localization in culture (PMID: 37030635), loss of DNA binding and decreased transactivation of Tp53 targets and interference with wild-type Tp53 transactivation, leads to resistance to apoptosis and failure of G1 arrest in cell culture (PMID: 23538418, PMID: 16861262, PMID: 31395785), as well as increased Akt activation, Stat3 dependent migration, and enhanced tumor onset and growth in mouse models (PMID: 30107178). |
Associated Drug Resistance | |
Category Variants Paths |
TP53 mutant TP53 exon7 TP53 R248Q TP53 mutant TP53 inact mut TP53 R248Q |
Transcript | NM_000546.6 |
gDNA | chr17:g.7674220C>T |
cDNA | c.743G>A |
Protein | p.R248Q |
Source Database | RefSeq |
Genome Build | GRCh38/hg38 |
Transcript | gDNA | cDNA | Protein | Source Database | Genome Build |
---|---|---|---|---|---|
NM_001126114 | chr17:g.7674220C>T | c.743G>A | p.R248Q | RefSeq | GRCh38/hg38 |
NM_001126112.2 | chr17:g.7674220C>T | c.743G>A | p.R248Q | RefSeq | GRCh38/hg38 |
NM_000546.6 | chr17:g.7674220C>T | c.743G>A | p.R248Q | RefSeq | GRCh38/hg38 |
NM_000546.5 | chr17:g.7674220C>T | c.743G>A | p.R248Q | RefSeq | GRCh38/hg38 |
NM_001407262.1 | chr17:g.7674220C>T | c.743G>A | p.R248Q | RefSeq | GRCh38/hg38 |
NM_001407268.1 | chr17:g.7674220C>T | c.743G>A | p.R248Q | RefSeq | GRCh38/hg38 |
NM_001126113 | chr17:g.7674220C>T | c.743G>A | p.R248Q | RefSeq | GRCh38/hg38 |
NM_001126112.3 | chr17:g.7674220C>T | c.743G>A | p.R248Q | RefSeq | GRCh38/hg38 |
NM_000546 | chr17:g.7674220C>T | c.743G>A | p.R248Q | RefSeq | GRCh38/hg38 |
NM_001407270.1 | chr17:g.7674220C>T | c.743G>A | p.R248Q | RefSeq | GRCh38/hg38 |
NM_001407266.1 | chr17:g.7674220C>T | c.743G>A | p.R248Q | RefSeq | GRCh38/hg38 |
NM_001126113.2 | chr17:g.7674220C>T | c.743G>A | p.R248Q | RefSeq | GRCh38/hg38 |
NM_001126114.2 | chr17:g.7674220C>T | c.743G>A | p.R248Q | RefSeq | GRCh38/hg38 |
NM_001126113.3 | chr17:g.7674220C>T | c.743G>A | p.R248Q | RefSeq | GRCh38/hg38 |
NM_001126114.3 | chr17:g.7674220C>T | c.743G>A | p.R248Q | RefSeq | GRCh38/hg38 |
NM_001407264.1 | chr17:g.7674220C>T | c.743G>A | p.R248Q | RefSeq | GRCh38/hg38 |
NM_001126112 | chr17:g.7674220C>T | c.743G>A | p.R248Q | RefSeq | GRCh38/hg38 |
Molecular Profile | Indication/Tumor Type | Response Type | Therapy Name | Approval Status | Evidence Type | Efficacy Evidence | References |
---|---|---|---|---|---|---|---|
TP53 R248Q | ovarian cancer | sensitive | ReACp53 | Preclinical - Pdx & cell culture | Actionable | In a preclinical study, ReACp53 rescued p53 activity and decreased viability of ovarian cancer cells harboring TP53 R248Q in culture, and induced tumor regression in patient-derived ovarian cancer xenograft models harboring TP53 R248Q (PMID: 26748848). | 26748848 |
TP53 R248Q | ovarian cancer | sensitive | APR-246 + Cisplatin | Preclinical | Actionable | In a preclinical study, the combination of APR-246 and Platinol (cisplatin) worked synergistically to inhibit growth of a Platinol (cisplatin)-resistant ovarian cancer cell line harboring TP53 R248Q in culture and inhibited tumor growth in TP53 R248Q-carrying ovarian cancer cell line xenograft models (PMID: 26086967). | 26086967 |
TP53 R248Q | Advanced Solid Tumor | sensitive | Ganetespib | Preclinical | Actionable | In a preclinical study, Ganetespib extended survival by 48% in mice with tumors expressing TP53 R248Q (PMID: 26009011). | 26009011 |
TP53 R248Q | acute myeloid leukemia | resistant | ALRN-6924 | Preclinical - Cell culture | Actionable | In a preclinical study, ALRN-6924 did not inhibit the growth of acute myeloid leukemia cells harboring TP53 R248Q in culture (PMID: 29643228). | 29643228 |
TP53 R248Q | colorectal cancer | sensitive | Tanespimycin | Preclinical | Actionable | In a preclincal study, Tanespimycin (17-AAG) treatment reduced p-Stat3 expression, induced apoptosis, and inhibited tumor growth of mutagen induced colorectal cancer in a genetic mouse model expressing TP53 R248Q (PMID: 30107178). | 30107178 |
TP53 R248Q | colorectal cancer | predicted - sensitive | Napabucasin | Preclinical - Cell culture | Actionable | In a preclinical study, Napabucasin (BBI608) treatment reduced Stat3 phosphorylation and impaired wound healing in colorectal cancer cell lines expressing TP53 R248Q in culture (PMID: 30107178). | 30107178 |
TP53 R248Q | acute myeloid leukemia | predicted - sensitive | thioureidobutyronitrile | Preclinical - Cell culture | Actionable | In a preclinical study, Kevetrin (thioureidobutyronitrile) treatment resulted in apoptosis, decreased cell viability, elevated expression of Tp53 target genes, and increased Tp53 nuclear localization in an acute myeloid leukemia cell line harboring TP53 R248Q in culture (PMID: 32945487). | 32945487 |