The Saccharomyces cerevisiae protein, PMR1, encodes P-type calcium transport ATPase, which localizes to the Golgi and regulates the intracellular transport of calcium and manganese. The human homologue, ATP2C1 (also designated SPLA in rat), also regulates the transport of calcium in the Golgi complex and is related to other P-type ATPases family members, such as the sarco(endo)plasmic calcium ATPase (SERCA) and the plasma membrane calcium ATPase (PCMA). PMR1 is a transmembrane protein that exists as two splice variants, which vary by 20 amino acids. PMR1 is mutated in Hailey-Hailey disease (HHD), which is an autosomal dominant disorder that is characterized by blisters and erosions of the skin. These findings provide further evidence that PMR1 plays a key role in maintaining the integrity of the epidermis by controlling intracellular calcium signaling.