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Review
. 2006 Oct;1762(10):906-19.
doi: 10.1016/j.bbadis.2006.08.010. Epub 2006 Sep 1.

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

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Free article
Review

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

Seasson N Phillips et al. Biochim Biophys Acta. 2006 Oct.
Free article

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.

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