MOLECULAR BIOLOGY OF THE NUERONAL CEROID-LIPOFUSCINOSES

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This subproject is one of many research subprojects utilizing the resources provided by a Center grant funded by NIH/NCRR. The subproject and investigator (PI) may have received primary funding from another NIH source, and thus could be represented in other CRISP entries. The institution listed is for the Center, which is not necessarily the institution for the investigator. Research is being conducted to determine the molecular basis of and to develop animal models for the hereditary neuronal ceroid lipofuscinoses ( NCLs). The NCLs are a group of similar fatal neurodegenerative disorders. The genes that are defective in several forms of NCL have been identified. We are using gene targeting to develop mouse gene knockout models for two forms of NCL. These mouse models will be used to study the mechanisms by which the gene defects lead to the disease pathology. We will use the supercomputing resource primarily for genetic database analyses and to design experiments based on these analyses.