Chaired by:
Constantine A. Stratakis, NICHD

Balcony B, Natcher Conference Center

The cloning of the Carney complex (CNC) PRKAR1A gene, protein kinase A (PKA) and its signaling pathway have been linked multiple times to human disease, beginning with the identification of GNAS as the gene responsible for McCune Albright syndrome (MAS) and ending with the latest addition to the c-AMP signaling and tumorigenesis story. PKA is central to many cellular processes including growth, proliferation, and metabolism. The presence of inactivating germline mutations and the loss of its wild type allele in CNC lesions indicated that PRKAR1A could function as a tumor–suppressor gene in these tissues. However, there are conflicting data in the literature about PRKAR1A's role in cancer cell lines, in human neoplasms, and in animal models. GNAS appears to function as a classic oncogene. PKA regulates fat cell metabolism and a novel protein, perilipin. In this session, we review briefly the genetics of MAS and CNC and focus on PKA's involvement in human tumorigenesis and fat cell metabolism.

Program

McCune-Albright Syndrome, GNAS Mutations and Carney Complex
Michael Collins, NIDCR

Protein Kinase A in Endocrine Tumors
Constantine A. Stratakis, NICHD

Protein Kinase A in Human Cancer: Transcriptional and Translational Strategies for Silencing Gene Expression
Yoon Cho-Chung, NCI

Perilipin, a Critical Regulator of Adipose Lipid Storage and Metabolism
Dean Londos, NIDDK