We describe the development of human renal angiomyolipoma (AML) cell lines with which to provide an in vitro cellular model for use in identifying molecular markers of Tuberous Sclerosis Complex (TSC). Specifically, one goal of this proposal is to immortalize human AML cells and generate a set of matching TSC gene knock-in control cell lines. We shall use these cell-line reagents for whole cell biopanning using a naive bacteriophage M13 antibody library. AML specific markers will be validated using TSC-affected, and normal tissue samples. This proposal will form the foundation to a series of studies to identify cell markers that result from the inactivation of either the TSC1 or TSC2 genes. The current therapy for TSC is surgery to remove the lesions that result as a consequence of the disease. This option is not available for some clinical manifestations. The ultimate goal of our studies will be to derive a human monoclonal antibody for the immunotherapeutic treatment of TSC that can be used for all TSC patients. ? ? ?
