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The tyrosine phosphatase Shp2 in development and cancer

Item Type:Review
Title:The tyrosine phosphatase Shp2 in development and cancer
Creators Name:Grossmann, K.S., Rosario, M., Birchmeier, C. and Birchmeier, W.
Abstract:Deregulation of signaling pathways, through mutation or other molecular changes, can ultimately result in disease. The tyrosine phosphatase Shp2 has emerged as a major regulator of receptor tyrosine kinase (RTK) and cytokine receptor signaling. In the last decade, germline mutations in the human PTPN11 gene, encoding Shp2, were linked to Noonan (NS) and LEOPARD syndromes, two multisymptomatic developmental disorders that are characterized by short stature, craniofacial defects, cardiac defects, and mental retardation. Somatic Shp2 mutations are also associated with several types of human malignancies, such as the most common juvenile leukemia, juvenile myelomonocytic leukemia (JMML). Whereas NS and JMML are caused by gain-of-function (GOF) mutations of Shp2, loss-of-function (LOF) mutations are thought to be associated with LEOPARD syndrome. Animal models that carry conditional LOF and GOF mutations have allowed a better understanding of the mechanism of Shp2 function in disease, and shed light on the role of Shp2 in signaling pathways that control decisive events during embryonic development or during cellular transformation/tumorigenesis.
Keywords:Morphogenesis, Neoplasms, Non-Receptor Type 11 Protein Tyrosine Phosphatase, Signal Transduction, Animals
Source:Advances in Cancer Research
Publisher:Academic Press
Page Range:53-89
Official Publication:https://doi.org/10.1016/S0065-230X(10)06002-1
PubMed:View item in PubMed

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