Tau protein, a kind of microtubule?associated protein, plays an important role in the pathogenesis of Alzheimer’s disease (AD) and other tauopathies, and has drawn more and more attention of scientific researchers.Appropriate animal models of mutated tau protein are important in the studies of the pathogenesis and drug therapy of tauopathies. So far, some transgenic animal models expressing human tau protein have been established, among which the transgenic animal model overexpressing P301L mutated form of tau protein has been widely used because of its obvious pathological changes. In this review, we will review the research progress in the pathological manifestations of the P301L tau transgenic animal model and its applications in pharmacological studies.