title: Unraveling protein misfolding diseases using model systems.
abstract: Experimental model systems have long been used to probe the causes, consequences and mechanisms of pathology leading to human disease. Ideally, such information can be exploited to inform the development of therapeutic strategies or treatments to combat disease progression. In the case of protein misfolding diseases, a wide range of model systems have been developed to investigate different aspects of disorders including Huntington's disease, Parkinson's disease, Alzheimer's disease as well as amyotrophic lateral sclerosis. Utility of these systems broadly correlates with evolutionary complexity: small animal models such as rodents and the fruit fly are appropriate for pharmacological modeling and cognitive/behavioral assessment, the roundwormCaenorhabditis elegansallows analysis of tissue-specific disease features, and unicellular organisms such as the yeastSaccharomyces cerevisiaeand the bacteriumEscherichia coliare ideal for molecular studies. In this chapter, we highlight key advances in our understanding of protein misfolding/unfolding disease provided by model systems.
authors.affiliation: University of Texas Graduate School of Biomedical Sciences, Houston, TX 77030, USA; University of Texas Graduate School of Biomedical Sciences, Houston, TX 77030, USA.
authors.forename: Sara
authors.initials: S
authors.lastname: Peffer
authors.affiliation: University of Texas Graduate School of Biomedical Sciences, Houston, TX 77030, USA; University of Texas Graduate School of Biomedical Sciences, Houston, TX 77030, USA.
authors.forename: Kimberly
authors.initials: K
authors.lastname: Cope
authors.affiliation: Department of Microbiology & Molecular Genetics, University of Texas Medical School, Houston, TX 77030, USA; Department of Microbiology & Molecular Genetics, University of Texas Medical School, Houston, TX 77030, USA.
authors.forename: Kevin A
authors.initials: KA
authors.lastname: Morano