TY - JOUR
T1 - Ménétrier disease and gastrointestinal stromal tumors
T2 - Hyperproliferative disorders of the stomach
AU - Coffey, Robert J.
AU - Washington, Mary Kay
AU - Corless, Christopher L.
AU - Heinrich, Michael C.
PY - 2007/1/4
Y1 - 2007/1/4
N2 - Ménétrier disease and gastrointestinal stromal tumors (GISTs) are hyperproliferative disorders of the stomach caused by dysregulated receptor tyrosine kinases (RTKs). In Ménétrier disease, overexpression of TGF-α, a ligand for the RTK EGFR, results in selective expansion of surface mucous cells in the body and fundus of the stomach. In GISTs, somatic mutations of the genes encoding the RTK KIT (or PDGFRA in a minority of cases) result in constitutive kinase activity and neoplastic transformation of gut pacemaker cells (interstitial cells of Cajal). On the basis of the involvement of these RTKs in the pathogenesis of these disorders, Ménétrier disease patients have been effectively treated with a blocking monoclonal antibody specific for EGFR and GIST patients with KIT and PDGFRA tyrosine kinase inhibitors.
AB - Ménétrier disease and gastrointestinal stromal tumors (GISTs) are hyperproliferative disorders of the stomach caused by dysregulated receptor tyrosine kinases (RTKs). In Ménétrier disease, overexpression of TGF-α, a ligand for the RTK EGFR, results in selective expansion of surface mucous cells in the body and fundus of the stomach. In GISTs, somatic mutations of the genes encoding the RTK KIT (or PDGFRA in a minority of cases) result in constitutive kinase activity and neoplastic transformation of gut pacemaker cells (interstitial cells of Cajal). On the basis of the involvement of these RTKs in the pathogenesis of these disorders, Ménétrier disease patients have been effectively treated with a blocking monoclonal antibody specific for EGFR and GIST patients with KIT and PDGFRA tyrosine kinase inhibitors.
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U2 - 10.1172/JCI30491
DO - 10.1172/JCI30491
M3 - Review article
C2 - 17200708
AN - SCOPUS:33846014993
SN - 0021-9738
VL - 117
SP - 70
EP - 80
JO - Journal of Clinical Investigation
JF - Journal of Clinical Investigation
IS - 1
ER -