期刊
FEBS LETTERS
卷 580, 期 10, 页码 2477-2482出版社
WILEY
DOI: 10.1016/j.febslet.2006.03.088
关键词
LEOPARD syndrome; PTPN11; SHP-2 phosphatase activity; Gab1; PI3K
LEOPARD (LS) and Noonan (NS) are overlapping syndromes associated with distinct mutations of SHP-2. Whereas NS mutations enhance SHP-2 catalytic activity, we show that the activity of three representative LS mutants is undetectable when assayed using a standard protein tyrosine phosphatase (PTP) substrate. A different assay using a specific SHP-2 substrate confirms their decreased PTP activity, but also reveals a significant activity of the T468M mutant. In transfected cells stimulated with epidermal growth factor, the least active LS mutants promote Gab1/PI3K binding, validating our in vitro data. LS mutants thus display a reduced PTP activity both in vitro and in transfected cells. (c) 2006 Federation of European Biochemical Societies. Published by Elsevier B.V. All rights reserved.
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