Calcium in the Golgi apparatus.

Abstract The secretory-pathway Ca 2+ -ATPases (SPCAs) represent a recently recognized family of phosphorylation-type ATPases that supply the lumen of the Golgi apparatus with Ca 2+ and Mn 2+ needed for the normal functioning of this structure. Mutations of the human SPCA1 gene ( ATP2C1 ) cause Hailey–Hailey disease, an autosomal dominant skin disorder in which keratinocytes in the suprabasal layer of the epidermis detach. We will first review the physiology of the SPCAs and then discuss how mutated SPCA1 proteins can lead to an epidermal disorder.