Lysosomal Ion Channels and Human Diseases

Ion channels are a group of proteins residing in both the plasma membrane and intracellular membranes. Their opening allows the passage of ions from one side of the membrane to the other following the electrochemical gradient. The physiology and functions of ion channels in the plasma membrane have been extensively studied. Because of the inaccessibility of intracellular membranes, our understanding of organellular ion channels is largely hampered. However, new developments in biomedical methodology in recent years have made it possible to investigate intracellular ion channels. In this chapter, we provide a summary of newly identified ion channels in the lysosome. The characteristics, physiological functions, and pathological roles of these ion channels are discussed. We hope to guide the readers into a more in-depth discussion of the importance of lysosomal ion channels in cellular functions and human diseases.