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Gene | KIT |
Variant | S476I |
Impact List | missense |
Protein Effect | gain of function |
Gene Variant Descriptions | KIT S476I lies within the Ig-like C2-type domain 5 (exon 9) of the Kit protein (UniProt.org). S476I confers a gain of function on Kit, as indicated by constitutive phosphorylation of Kit in cell culture (PMID: 19865100, PMID: 30094412). |
Associated Drug Resistance | |
Category Variants Paths |
KIT mutant KIT act mut KIT S476I KIT mutant KIT exon9 KIT S476I |
Transcript | NM_000222.3 |
gDNA | chr4:g.54725937G>T |
cDNA | c.1427G>T |
Protein | p.S476I |
Source Database | RefSeq |
Genome Build | GRCh38/hg38 |
Transcript | gDNA | cDNA | Protein | Source Database | Genome Build |
---|---|---|---|---|---|
NM_000222 | chr4:g.54725937G>T | c.1427G>T | p.S476I | RefSeq | GRCh38/hg38 |
NM_000222.2 | chr4:g.54725937G>T | c.1427G>T | p.S476I | RefSeq | GRCh38/hg38 |
NM_001385286.1 | chr4:g.54725937G>T | c.1427G>T | p.S476I | RefSeq | GRCh38/hg38 |
NM_001093772 | chr4:g.54725937G>T | c.1427G>T | p.S476I | RefSeq | GRCh38/hg38 |
NM_001093772.1 | chr4:g.54725937G>T | c.1427G>T | p.S476I | RefSeq | GRCh38/hg38 |
NM_000222.3 | chr4:g.54725937G>T | c.1427G>T | p.S476I | RefSeq | GRCh38/hg38 |
XM_017008178.1 | chr4:g.54725937G>T | c.1427G>T | p.S476I | RefSeq | GRCh38/hg38 |
NM_001093772.2 | chr4:g.54725937G>T | c.1427G>T | p.S476I | RefSeq | GRCh38/hg38 |
NM_001385285.1 | chr4:g.54725937G>T | c.1427G>T | p.S476I | RefSeq | GRCh38/hg38 |
XM_017008178 | chr4:g.54725937G>T | c.1427G>T | p.S476I | RefSeq | GRCh38/hg38 |
XM_017008180 | chr4:g.54725937G>T | c.1427G>T | p.S476I | RefSeq | GRCh38/hg38 |
XM_017008180.1 | chr4:g.54725937G>T | c.1427G>T | p.S476I | RefSeq | GRCh38/hg38 |
Molecular Profile | Indication/Tumor Type | Response Type | Therapy Name | Approval Status | Evidence Type | Efficacy Evidence | References |
---|---|---|---|---|---|---|---|
KIT S476I | mast-cell leukemia | predicted - sensitive | Midostaurin | Preclinical - Patient cell culture | Actionable | In a preclinical study, treatment with Rydapt (midostaurin) decreased proliferation of patient-derived neoplastic mast cells harboring KIT S476I in culture (PMID: 25209843). | 25209843 |
KIT S476I | mast-cell leukemia | predicted - sensitive | Nilotinib | Preclinical - Patient cell culture | Actionable | In a preclinical study, treatment with Tasigna (nilotinib) decreased proliferation of patient-derived neoplastic mast cells harboring KIT S476I in culture (PMID: 25209843). | 25209843 |