Chapter 18 Rodent Models of Autosomal Dominant Parkinson Disease Jianfeng Xiao1, Satya Vemula1, Zhenyu Yue2 1 Department of Neurology, University of Tennessee Health Science Center, Memphis, TN, USA 2 Departments of Neurology and Neuroscience, Icahn School of Medicine at Mount Sinai, New York, NY, USA Abstract Parkinson disease (PD) is a common and impactful neurodegenerative disorder that affects more than 1% of the human population over 60 years of age. Although most human cases are sporadic, familial cases (<10%) with autosomal dominant genetic components are not uncommon in clinical practice. Animal models of autosomal dominant PD help in understanding the pathogenic processes related to neuronal loss and facilitate development of novel pharmacological approaches to therapy. Decades after the identification of causal genes and the application of clinical genetic testing, and despite numerous established rodent models of PD, we are still lacking a suitable model that replicates all of the motor and nonmotor features seen in human PD. In this review, we summarize many of the published murine models of autosomal dominant PD and discuss the current status and value of genetically engineered PD rodent models in understanding disease mechanisms and therapeutics development.