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Gene | KIT |
Variant | P551_V555del |
Impact List | deletion |
Protein Effect | gain of function |
Gene Variant Descriptions | KIT P551_V555del results in the deletion of five amino acids in the juxtamembrane domain (exon 11) of the Kit protein from amino acids 551 to 555 (PMID: 9438854). P551_V555del confers a gain of function on Kit, as indicated by constitutive Kit phosphorylation, and is transforming in cell culture (PMID: 9438854). |
Associated Drug Resistance | |
Category Variants Paths |
KIT mutant KIT act mut KIT exon 11 del KIT P551_V555del KIT mutant KIT exon11 KIT exon 11 del KIT P551_V555del |
Transcript | NM_000222.3 |
gDNA | chr4:g.54727420_54727434del15 |
cDNA | c.1652_1666del15 |
Protein | p.P551_V555delPMYEV |
Source Database | RefSeq |
Genome Build | GRCh38/hg38 |
Transcript | gDNA | cDNA | Protein | Source Database | Genome Build |
---|---|---|---|---|---|
NM_000222.3 | chr4:g.54727420_54727434del15 | c.1652_1666del15 | p.P551_V555delPMYEV | RefSeq | GRCh38/hg38 |
XM_017008178.1 | chr4:g.54727420_54727434del15 | c.1652_1666del15 | p.P551_V555del | RefSeq | GRCh38/hg38 |
NM_001385290.1 | chr4:g.54727416_54727430del15 | c.1651_1665del15 | p.K551_E555delKPMYE | RefSeq | GRCh38/hg38 |
NM_001385284.1 | chr4:g.54727416_54727430del15 | c.1651_1665del15 | p.K551_E555delKPMYE | RefSeq | GRCh38/hg38 |
NM_000222.2 | chr4:g.54727420_54727434del15 | c.1652_1666del15 | p.P551_V555del | RefSeq | GRCh38/hg38 |
XM_017008178 | chr4:g.54727420_54727434del15 | c.1652_1666del15 | p.P551_V555del | RefSeq | GRCh38/hg38 |
NM_001385292.1 | chr4:g.54727429_54727443del15 | c.1652_1666del15 | p.E551_K555delEVQWK | RefSeq | GRCh38/hg38 |
NM_001385285.1 | chr4:g.54727420_54727434del15 | c.1652_1666del15 | p.P551_V555delPMYEV | RefSeq | GRCh38/hg38 |
NM_001385286.1 | chr4:g.54727433_54727447del15 | c.1653_1667del15 | p.Q552_V556delQWKVV | RefSeq | GRCh38/hg38 |
NM_001093772.2 | chr4:g.54727433_54727447del15 | c.1653_1667del15 | p.Q552_V556delQWKVV | RefSeq | GRCh38/hg38 |
NM_000222 | chr4:g.54727420_54727434del15 | c.1652_1666del15 | p.P551_V555del | RefSeq | GRCh38/hg38 |
NM_001385288.1 | chr4:g.54727429_54727443del15 | c.1652_1666del15 | p.E551_K555delEVQWK | RefSeq | GRCh38/hg38 |
Molecular Profile | Indication/Tumor Type | Response Type | Therapy Name | Approval Status | Evidence Type | Efficacy Evidence | References |
---|---|---|---|---|---|---|---|
KIT P551_V555del | gastrointestinal stromal tumor | sensitive | Sorafenib | Preclinical - Pdx | Actionable | In a preclinical study, Nexavar (sorafenib) inhibited Erk signaling, induced apoptosis, resulted in tumor growth inhibition in patient derived xenograft models of gastrointestinal stromal tumor harboring KIT P551_V555del (PMID: 19139124). | 19139124 |