SPCA1 pumps and Hailey–Hailey disease

Both the endoplasmic reticulum and the Golgi apparatus are agonist-sensitive intracellular Ca 2+ stores. The Golgi apparatus has Ca 2+-release channels and a Ca 2+-uptake mechanism consisting of sarco(endo)plasmic-reticulum Ca 2+-ATPases (SERCA) and secretory-pathway Ca 2+-ATPases (SPCA). SPCA1 has...

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Veröffentlicht in:Biochemical and biophysical research communications 2004-10, Vol.322 (4), p.1204-1213
Hauptverfasser: Missiaen, Ludwig, Raeymaekers, Luc, Dode, Leonard, Vanoevelen, Jo, Van Baelen, Kurt, Parys, Jan B., Callewaert, Geert, De Smedt, Humbert, Segaert, Siegfried, Wuytack, Frank
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container_end_page 1213
container_issue 4
container_start_page 1204
container_title Biochemical and biophysical research communications
container_volume 322
creator Missiaen, Ludwig
Raeymaekers, Luc
Dode, Leonard
Vanoevelen, Jo
Van Baelen, Kurt
Parys, Jan B.
Callewaert, Geert
De Smedt, Humbert
Segaert, Siegfried
Wuytack, Frank
description Both the endoplasmic reticulum and the Golgi apparatus are agonist-sensitive intracellular Ca 2+ stores. The Golgi apparatus has Ca 2+-release channels and a Ca 2+-uptake mechanism consisting of sarco(endo)plasmic-reticulum Ca 2+-ATPases (SERCA) and secretory-pathway Ca 2+-ATPases (SPCA). SPCA1 has been shown to transport both Ca 2+ and Mn 2+ in the Golgi lumen and therefore plays an important role in the cytosolic and intra-Golgi Ca 2+ and Mn 2+ homeostasis. Human genetic studies have provided new information on the physiological role of SPCA1. Loss of one functional copy of the SPCA1 ( ATP2C1) gene causes Hailey–Hailey disease, a skin disorder arising in the adult age with recurrent vesicles and erosions in the flexural areas. Here, we review recent experimental evidence showing that the Golgi apparatus plays a much more important role in intracellular ion homeostasis than previously anticipated.
doi_str_mv 10.1016/j.bbrc.2004.07.128
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source MEDLINE; Elsevier ScienceDirect Journals Complete
subjects Adult
Animals
Calcium - metabolism
Calcium pump
Calcium store
Calcium-Transporting ATPases - genetics
Calcium-Transporting ATPases - physiology
Dermatology
Female
Golgi apparatus
Golgi Apparatus - enzymology
Hailey–Hailey disease
Humans
Intracellular calcium
Manganese - metabolism
Pemphigus, Benign Familial - enzymology
Pemphigus, Benign Familial - genetics
Pemphigus, Benign Familial - pathology
Secretory pathway
title SPCA1 pumps and Hailey–Hailey disease
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