Abstract
Recent evidence highlights the functional importance of the Golgi apparatus as an agonist-sensitive intracellular Ca 2+ store. Besides Ca 2+-release channels and Ca 2+-binding proteins, the Golgi complex contains Ca 2+-uptake mechanisms consisting of the well-known sarco/endoplasmic reticulum Ca 2+-transport ATPases (SERCA) and the much less characterized secretory-pathway Ca 2+-transport ATPases (SPCA). SPCA supplies the Golgi compartments and, possibly, the more distal compartments of the secretory pathway with both Ca 2+ and Mn 2+ and, therefore, plays an important role in the cytosolic and intra-Golgi Ca 2+ and Mn 2+ homeostasis. Mutations in the human gene encoding the SPCA1 pump ( ATP2C1) resulting in Hailey–Hailey disease, an autosomal dominant skin disorder, are discussed.
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