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This seminar course will examine recent research aimed at clarifying the molecular basis of highly conserved and ubiquitous genome maintenance pathways. While this research reveals DNA repair functions in the cell, it also describes basic mechanisms that underlie distinct disease processes. Specific topics include, but are not limited to, the involvement of DNA repair in preventing mutagenesis, DNA damage responses, immunoglobulin gene diversification, Huntington's disease, recombination, ataxia telangiectasia, SCAN1, Cockayne syndrome, xeroderma pigmentosum, trichothiodystrophy, Faconi's anemia, and progeria.