<p>Neurodegeneration is one of the leading factor for death globally, affecting millions of people. Developing animal models are critical to understand biological processes and comprehend pathological hallmarks of neurodegenerative diseases. For decades, many animal models have served as excellent tools to determine the disease progression, develop diagnostic methods and design novel therapies against distinct pathologies. Here, we provide a comprehensive overview of both, mammalian and non-mammalian animal models, with a focus on three most common and aggressive neurodegenerative disorders: Alzheimer’s disease, Parkinson’s disease and Spinocerebellar ataxia-1. We highlight various approaches including transgene, gene transfer, and chemically-induced methods used to develop disease models. In particular, we discuss applications of both non-mammalian and mammalian contributions in research on neurodegeneration. It is exciting to learn the roles of animal models in disease pathomechanisms, identifying biomarkers and hence devising novel interventions to treat neuropathological conditions.</p>