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Calsequestrin: a well-known but curious protein in skeletal muscle
Authors:Jin Seok Woo  Seung Yeon Jeong  Ji Hee Park  Jun Hee Choi  Eun Hui Lee
Affiliation:1.Department of Physiology, David Geffen School of Medicine, UCLA, Los Angeles, CA 10833 USA ;2.Department of Physiology, College of Medicine, The Catholic University of Korea, Seoul, 06591 Korea ;3.Department of Biomedicine & Health Sciences, Graduate School, The Catholic University of Korea, Seoul, 06591 Korea
Abstract:Calsequestrin (CASQ) was discovered in rabbit skeletal muscle tissues in 1971 and has been considered simply a passive Ca2+-buffering protein in the sarcoplasmic reticulum (SR) that provides Ca2+ ions for various Ca2+ signals. For the past three decades, physiologists, biochemists, and structural biologists have examined the roles of the skeletal muscle type of CASQ (CASQ1) in skeletal muscle and revealed that CASQ1 has various important functions as (1) a major Ca2+-buffering protein to maintain the SR with a suitable amount of Ca2+ at each moment, (2) a dynamic Ca2+ sensor in the SR that regulates Ca2+ release from the SR to the cytosol, (3) a structural regulator for the proper formation of terminal cisternae, (4) a reverse-directional regulator of extracellular Ca2+ entries, and (5) a cause of human skeletal muscle diseases. This review is focused on understanding these functions of CASQ1 in the physiological or pathophysiological status of skeletal muscle.Subject terms: Physiology, Calcium and vitamin D, Diseases
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