14th International Workshop on Ataxia-Telangiectasia and ATM
Principal Investigator: Richard A Gatti
Abstract: Funds are requested for partial support towards the 14th International Workshop on Ataxia-Telangiectasia and ATM, which will be held at the Crowne Plaza Hotel in Redondo Beach, CA. on April 11-14, 2010. We expect to invite approximately 60 speakers;half of these will be selected by the Organizing Committee and Co-Chairs of their sessions, while the other half will come from abstracts submitted in response to an AT/ATM-focused list of "Topics of Interest" that will be posted on the conference website. The incoming abstracts will be reviewed by both the Organizing Committee and the Co-Chairs. The remaining abstracts will be presented as posters;there is room for 100 posters. Ataxia-telangiectasia (A-T) is a complex autosomal recessive disorder that is characterized by a progressive cerebellar ataxia, telangiectasia, immunodeficiency, genomic instability, radiation sensitivity, and a markedly increased incidence of cancer, usually lymphoma or leukemia. It has been estimated that 1-6% of the general population carries mutations in one allele of the ATM (ataxia-telangiectasia mutated) gene and ATM heterozygotes have an increased risk of developing breast cancer. The ATM gene encodes a large (369 kDa) protein, ATM, which is a member of the phosphatidyl inositol 3 kinase family of serine/threonine protein kinases. The major role of ATM appears to be in the recognition and repair of double strand DNA breaks, through coordinated cell cycle checkpoint control and signaling to hundreds of downstream targets. Cells that lack ATM are highly sensitive to ionizing radiation (IR) and other DNA damaging agents. Understanding the function of ATM is of considerable importance to understanding the role of ATM in neural development and neurodegeneration, the molecular basis for increased cancer predisposition, and the molecular mechanisms that control how cells respond to radiation damage and radiomimetic chemotherapeutic drugs. A-T has also become a model for the study of mutation-targeted drugs for correcting genetic diseases. Animal models and hundreds of patient-derived cell lines are available, with identified mutations. Objectives of the meeting: The objectives of the meeting are to bring together an international group of basic and clinical researchers working on various aspects of A-T and related neurodegenerative diseases, as well as on the role of ATM in cancer, the DNA damage response, and in viral infections that involve DNA integration. Our goal is to stimulate research that will lead to better understanding and treatment of A-T and other genetic diseases. Specific areas of focus of the meeting will include the role of ATM and related proteins in the DNA damage response, the importance of chromatin and genomic instability in the activation of ATM, the role of ATM in the developing nervous system and in neurodegeneration, animal models of A-T, ATM mutations in breast and other human cancers, the epidemiology of ATM mutations, and the development and design of future clinical trials for A-T patients worldwide. We also hope to encourage new young investigators to develop an interest in both clinical and basic A-T/ATM-related research.
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more information: NIH RePORT