title: Modeling human neurodegenerative diseases in transgenic systems.
abstract: Transgenic systems are widely used to study the cellular and molecular basis of human neurodegenerative diseases. A wide variety of model organisms have been utilized, including bacteria (Escherichia coli), plants (Arabidopsis thaliana), nematodes (Caenorhabditis elegans), arthropods (Drosophila melanogaster), fish (zebrafish, Danio rerio), rodents (mouse, Mus musculus and rat, Rattus norvegicus) as well as non-human primates (rhesus monkey, Macaca mulatta). These transgenic systems have enormous value for understanding the pathophysiological basis of these disorders and have, in some cases, been instrumental in the development of therapeutic approaches to treat these conditions. In this review, we discuss the most commonly used model organisms and the methodologies available for the preparation of transgenic organisms. Moreover, we provide selected examples of the use of these technologies for the preparation of transgenic animal models of neurodegenerative diseases, including Alzheimer's disease (AD), frontotemporal lobar degeneration (FTLD), amyotrophic lateral sclerosis (ALS), Huntington's disease (HD) and Parkinson's disease (PD) and discuss the application of these technologies to AD as an example of how transgenic modeling has affected the study of human neurodegenerative diseases.
authors.affiliation: Research and Development Service, James J. Peters Department of Veterans Affairs Medical Center, Bronx, NY 10468, USA. miguel.gama-sosa@mssm.edu
authors.forename: Miguel A
authors.initials: MA
authors.lastname: Gama Sosa
authors.forename: Rita
authors.initials: R
authors.lastname: De Gasperi
authors.forename: Gregory A
authors.initials: GA
authors.lastname: Elder
mesh_terms: D000544:Alzheimer Disease / Q000235:genetics / Q000628:therapy; D016564:Amyloid beta-Protein Precursor / Q000235:genetics; D000818:Animals; D030801:Animals, Genetically Modified; D004195:Disease Models, Animal; D006801:Humans; D000071058:Huntingtin Protein; D006816:Huntington Disease / Q000235:genetics / Q000628:therapy; D009419:Nerve Tissue Proteins / Q000235:genetics; D019636:Neurodegenerative Diseases / Q000235:genetics* / Q000628:therapy; D009687:Nuclear Proteins / Q000235:genetics; D019076:Transgenes / Q000235:genetics*
chemical_list: D016564:Amyloid beta-Protein Precursor; C086055:HTT protein, human; D000071058:Huntingtin Protein; D009419:Nerve Tissue Proteins; D009687:Nuclear Proteins