Funds are requested for partial support of the Seventh International Conference on the Neuronal Ceroid-Lipofuscinoses (NCL). This conference has become the primary forum for the presentation of new data on NCL research. Neuronal ceroid-lipofuscinoses (Batten Disease) are a group of inherited, progressive neurological disorders characterized by motor sensory deterioration, visual failure, severe mental retardation, and seizures. The pathological hallmark of this disorder is the accumulation of autofluorescent lipopigments known as ceroids in the brain and other tissues. They are very common neurogenetic disorders affecting infants and children. Some estimates indicate that in the United States, the disorders are more prevalent than all the sphingolipidoses combined. Although the disease entities have been recognized for more than 100 years, the biochemical defects remain largely unknown and no treatments are available. The previous six international conferences on this subject have sparked much collaborative research activity in this field bringing together researchers from across the world. Currently rapid progress is being made in understanding the biochemical defects, genetic localizations, gene organizations and molecular diagnosis of these disorders. The planned conference will bring together scientists of different disciplines and research interests to enhance information about these advances with a particular emphasis on understanding the pathogenesis of the disorders. The funds requested are specifically for the travel and lodging expenses of the invited speakers.
