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GIST: overview and<br />
molecular biology<br />
* protooncogene c - Kit encodes<br />
transmembrane tyros<strong>in</strong>e k<strong>in</strong>ase<br />
receptor located on long arm of<br />
chr 4 (4q11 - q12);<br />
A) dimerization and activation of<br />
normal c - Kit receptor by its<br />
ligand stem - cell factor;<br />
B) <strong>in</strong> gastro<strong>in</strong>test<strong>in</strong>al stromal<br />
tumors (<strong>GISTs</strong>), <strong>in</strong> - frame<br />
deletions and po<strong>in</strong>t mutations <strong>in</strong> c<br />
- Kit produce ligand - <strong>in</strong>dependent<br />
constitutive activation of c - Kit;<br />
[platelet derived growth factor α<br />
(PDGFα) receptor operational <strong>in</strong><br />
pathogenesis of <strong>GISTs</strong> and<br />
dermatofibrosarcoma<br />
protuberans]<br />
ATP, adenos<strong>in</strong>e triphosphate; ADP,<br />
adenos<strong>in</strong>e diphosphate; P, phosphate.