Annotation Detail

Information
Associated Genes
KIT
Associated Variants
KIT p.Asp817Glu (p.D817E) ( ENST00000288135.6, ENST00000412167.7, ENST00000686011.1, ENST00000687109.1, ENST00000687246.1, ENST00000687295.1, ENST00000689832.1, ENST00000689994.1, ENST00000690543.1, ENST00000692783.1 )
KIT p.Asp817Glu (p.D817E) ( ENST00000288135.6, ENST00000412167.7, ENST00000686011.1, ENST00000687109.1, ENST00000687246.1, ENST00000687295.1, ENST00000689832.1, ENST00000689994.1, ENST00000690543.1, ENST00000692783.1 )
Associated Disease
gastrointestinal stromal tumor
Source Database
CIViC Evidence
Description
Several preclinical studies were done in order to test the effects of various tyrosine kinase inhibitors (TKIs) on KIT-mutant cell lines derived from Gastrointestinal Stromal Tumor (GIST) patients with primary mutation 560-578 deletion and secondary mutation D816E. The cells were tested for Imatinib and Sunitinib resistances. GIST-T1/816 cells with 560-578 deletion and D816E mutations were resistant towards Imatinib and Sunitinib (Imatinib IC50: 604 nM and Sunitinib IC50: 3111 nM) compared to GIST-T1 cells with the 560-578 deletion mutation alone (Imatinib IC50: 30 nM and Sunitinib IC50: 15 nM). Imatinib inhibited phosphorylation in KIT and ERK at high concentrations. Sunitinib did not inhibit phosphorylation in KIT, ERK, or AKT at all. Additionally, Ba/F3 cells were mutagenized with N-ethyl-N-nitrosourea and incubated with varying concentrations of Sunitinib and Imatinib separately. In Imatinib incubated cells, D816E mutations were seen in less than 5% of resistant Ba/F3 cell clones only in the lowest concentration. In Imatinib incubated cells, D816E mutations were seen in approximately 10% of resistant Ba/F3 cell clones.
Variant Origin
somatic
Variant Origin
Somatic
Evidence URL
https://civic.genome.wustl.edu/links/evidence_items/4631
Gene URL
https://civic.genome.wustl.edu/links/genes/29
Variant URL
https://civic.genome.wustl.edu/links/variants/1559
Rating
4
Evidence Type
Predictive
Disease
Gastrointestinal Stromal Tumor
Evidence Direction
Supports
Drug
Sunitinib,Imatinib
Evidence Level
D
Clinical Significance
Resistance
Pubmed
25239608
Drugs
Drug NameSensitivitySupported
ImatinibResitance or Non-Reponsetrue
SunitinibResitance or Non-Reponsetrue