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Pathway : Renal cell carcinoma

Identifier  05211 Description  Renal cell cancer RCC accounts for ~3% of human malignancies and its incidence appears to be rising Although most cases of RCC seem to occur sporadically, an inherited predisposition to renal cancer accounts for 1-4% of cases RCC is not a single disease, it has several morphological subtypes Conventional RCC clear cell RCC accounts for ~80% of cases, followed by papillary RCC 10-15%, chromophobe RCC 5%, and collecting duct RCC <1% Genes potentially involved in sporadic neoplasms of each particular type are VHL, MET, BHD, and FH respectively In the absence of VHL, hypoxia-inducible factor alpha HIF-alpha accumulates, leading to production of several growth factors, including vascular endothelial growth factor and platelet-derived growth factor Activated MET mediates a number of biological effects including motility, invasion of extracellular matrix, cellular transformation, prevention of apoptosis and metastasis formation Loss of functional FH leads to accumulation of fumarate in the cell, triggering inhibition of HPH and preventing targeted pVHL-mediated degradation of HIF-alpha BHD mutations cause the Birt-Hogg-Dube syndrome and its associated chromophobe, hybrid oncocytic, and conventional clear cell RCC

1 Data Sets

141 Genes

0 Proteins