The blood-brain barrier is a network of endothelial cells that are tightly attached with each other via specialized cell-cell contacts. This passive diffusion barrier is complemented by ATP-binding cassette (ABC) transporters, which are localized on the surface of the endothelial cells. ABC transporters play important roles in the maintenance of blood-brain barrier integrity, as they carry a wide range of organic molecules, cell metabolites, and nutrients both out of the brain and into the brain. Recent studies have unraveled important roles of ABC transporters in the preservation of tissue homeostasis, pointing out the fact that ABC transporters protect both brain parenchymal cells and microvascular cells from injury. As such, ABC transporters have been involved in the pathogenesis of age-related neurodegenerative diseases, such as Parkinson and Alzheimer diseases, recently. This has led to the idea that neurodegenerative processes might be targeted by restoration of transport processes across the blood-brain barrier.