Characterizing pathogenic processes in Batten disease: Use of small eukaryotic model systems

Center for Aging and Developmental Biology, Aab Institute of Biomedical Science, University of Rochester School of Medicine and Dentistry, Rochester, NY 14642, USA.
Biochimica et Biophysica Acta (Impact Factor: 4.66). 11/2006; 1762(10):906-19. DOI: 10.1016/j.bbadis.2006.08.010
Source: PubMed

ABSTRACT The neuronal ceroid lipofuscinoses (NCLs) are neurodegenerative disorders. Nevertheless, small model organisms, including those lacking a nervous system, have proven invaluable in the study of mechanisms that underlie the disease and in studying the functions of the conserved proteins associated to each disease. From the single-celled yeast, Saccharomyces cerevisiae and Schizosaccharomyces pombe, to the worm, Caenorhabditis elegans and the fruitfly, Drosophila melanogaster, biochemical and, in particular, genetic studies on these organisms have provided insight into the NCLs.