Characterizing pathogenic processes in Batten disease: Use of small eukaryotic model systems
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- 作者：Seasson N. Phillips ; Neda Muzaffar ; Sandra Codlin ; Christopher A. Korey ; Peter E.M. Taschner ; Gert de Voer ; Sara E. Mole and David A. Pearce
- 关键词：Batten disease ; Model system ; NCL
- 刊名：Biochimica et Biophysica Acta (BBA)/Molecular Basis of Disease
- 出版年：2006
- 出版时间：October 2006
- 年：2006
- 卷：1762
- 期：10
- 页码：906-919
- 全文大小：1795 K
文摘
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.