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Gene | KIT |
Variant | W557G |
Impact List | missense |
Protein Effect | gain of function - predicted |
Gene Variant Descriptions | KIT W557G lies within the juxtamembrane domain (exon 11) of the Kit protein (PMID: 16226710). W557G results in constitutive phosphorylation of Kit in cell culture (PMID: 23567324), and therefore, is predicted to lead to a gain of Kit protein function. |
Associated Drug Resistance | |
Category Variants Paths |
KIT mutant KIT act mut KIT W557G KIT mutant KIT exon11 KIT W557G |
Transcript | NM_000222.3 |
gDNA | chr4:g.54727437T>G |
cDNA | c.1669T>G |
Protein | p.W557G |
Source Database | RefSeq |
Genome Build | GRCh38/hg38 |
Transcript | gDNA | cDNA | Protein | Source Database | Genome Build |
---|---|---|---|---|---|
NM_001385285.1 | chr4:g.54727437T>G | c.1669T>G | p.W557G | RefSeq | GRCh38/hg38 |
NM_000222.3 | chr4:g.54727437T>G | c.1669T>G | p.W557G | RefSeq | GRCh38/hg38 |
XM_017008178 | chr4:g.54727437T>G | c.1669T>G | p.W557G | RefSeq | GRCh38/hg38 |
NM_000222 | chr4:g.54727437T>G | c.1669T>G | p.W557G | RefSeq | GRCh38/hg38 |
NM_000222.2 | chr4:g.54727437T>G | c.1669T>G | p.W557G | RefSeq | GRCh38/hg38 |
XM_017008178.1 | chr4:g.54727437T>G | c.1669T>G | p.W557G | RefSeq | GRCh38/hg38 |
Molecular Profile | Indication/Tumor Type | Response Type | Therapy Name | Approval Status | Evidence Type | Efficacy Evidence | References |
---|---|---|---|---|---|---|---|
KIT W557G | extraskeletal myxoid chondrosarcoma | predicted - sensitive | Imatinib | Case Reports/Case Series | Actionable | In a clinical case study, Gleevec (imatinib) treatment resulted in stable disease lasting at least 3 years in a patient with extraskeletal myxoid chondrosarcoma harboring KIT W557G (PMID: 34446510). | 34446510 |
KIT W557G | Advanced Solid Tumor | sensitive | Imatinib | Preclinical | Actionable | In a preclinical study, Gleevec (imatinib mesylate) inhibited Kit phosphorylation efficiently in transformed human cells over expressing KIT W557G in culture (PMID: 23567324). | 23567324 |