Transgenic and physiological mouse models give insights into different aspects of amyotrophic lateral sclerosis

摘要: A wide range of genetic mouse models is available to help researchers dissect human disease mechanisms. Each type model has its own distinctive characteristics arising from the nature introduced mutation, as well specific changes gene interest. Here, we review current with mutations in genes causative for neurodegenerative amyotrophic lateral sclerosis. We focus on two main types mutants: transgenic mice and those that express mutant at physiological levels targeting or chemical mutagenesis. compare phenotypes which classes exist, illustrate what they can teach us about different aspects disease, noting informative may not necessarily mimic full trajectory condition. Transgenic greatly overexpress wild-type proteins, giving insight into protein deposition mechanisms, whereas expressing develop slowly progressing but early-stage processes. Although no fully recapitulate condition, almost all understand normal abnormal biological processes, providing individual each type, how these affect interpretation data generated model, are considered appreciated.