Annotation Detail

Information
Associated Genes
KIT
Associated Variants
KIT p.Ala830Pro (p.A830P) ( ENST00000288135.6, ENST00000412167.7, ENST00000686011.1, ENST00000687109.1, ENST00000687246.1, ENST00000687295.1, ENST00000689832.1, ENST00000689994.1, ENST00000690543.1, ENST00000692783.1 )
KIT p.Ala830Pro (p.A830P) ( 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 tested the effects of various tyrosine kinase inhibitors (TKIs) on Ba/F3 cell lines with representative primary sunitinib and imatinib sensitizing mutation del 557-558, which induced IL-3 independent growth, and secondary mutation A829P. The cells were tested for Sunitinib and Imatinib resistances. Ba/F3 cells with del 557-558 and A829P mutations did show resistance towards Sunitinib and Imatinib (Sunitinib IC50 for growth inhibition: 319 +- 88 nM and Imatinib IC50: 179 +- 27 nM) compared to Ba/F3 cells with the del 557-558 mutation (Sunitinib IC50: 7 +- 2 nM and Imatinib IC50: 27 +- 8 nM). Imatinib and Sunitinib were shown to not reduce p-KIT at all in Ba/F3 cells with del 557-558/ A829P mutations. Patient-derived GIST-T1/829 cells with 560-578 deletion/A829P mutations (Sunitinib IC50: 1168 nM and Imatinib IC50:1201 nM) did show resistance towards Sunitinib and Imatinib compared to GIST-T1 cells with 560-578 deletion alone (Sunitinib IC50: 15 nM and Imatinib IC50: 30 nM). Sunitinib and Imatinib did not inhibit p-KIT, p-ERK, and p-AKT in GIST-T1/829 cells.
Variant Origin
somatic
Variant Origin
Somatic
Evidence URL
https://civic.genome.wustl.edu/links/evidence_items/7415
Gene URL
https://civic.genome.wustl.edu/links/genes/29
Variant URL
https://civic.genome.wustl.edu/links/variants/990
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