期刊
CELL CALCIUM
卷 41, 期 5, 页码 405-416出版社
ELSEVIER SCI LTD
DOI: 10.1016/j.ceca.2006.11.001
关键词
Golgi apparatus; calcium; SPCA; Hailey-Hailey disease
类别
The secretory-pathway Ca2+-ATPases (SPCAs) represent a recently recognized family of phosphorylation-type ATPases that supply the lumen of the Golgi apparatus with Ca2+ and Mn2+ needed for the normal functioning of this structure. Mutations of the human SPCA1 gene (ATP2C1) cause Hailey-Hailey disease, an amosomal 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. (c) 2006 Elsevier Ltd. All rights reserved.
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