Article
Reduced phosphatase activity of SHP-2 in LEOPARD syndrome: consequences for PI3K binding on Gab1.
FEBS letters - 1 May 2006
Hanna Nadine, Montagner Alexandra, Lee Wen Hwa, Miteva Maria, Vidal Michel, Vidaud Michel, Parfait Béatrice, Raynal Patrick
Abstract excerpt
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...
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