Sigma receptors are usually classified as a separate class of intracellular receptors. Among them sigma-1 receptor has received the most study in respect to pharmacology application. This receptor with average or high affinity binds a wide range of chemical compounds of very different structural classes and a variety of therapeutic and pharmacological properties. The sigma-1 receptor is a trans-membrane protein placed in the endoplasmic reticulum (ER), which regulates the function of inositol-3-phosphate receptor, stabilizing the calcium signaling between ER and mitochondria. The receptor in question is involved in the formation of many neurological and psychiatric states. It is assumed that the sigma-1 receptor acts as a sensor of normal calcium operation. The studies over the recent years have shown the role of a disturbance in calcium signaling in the pathogenesis of Alzheimer’s and huntington’s diseases. In particular, changes in calcium homeostasis of the endoplasmic reticulum lead to the break of synaptic connections in the neurons. Thus, sigma-1 receptor holds promise in application as potential therapeutic targets for the treatment of neuropathological diseases.