Calsequestrin-1

Definition:

Calsequestrin is a high-capacity, moderate affinity, calcium-binding protein and thus acts as an internal calcium store in muscle. Calcium ions are bound by clusters of acidic residues at the protein surface, often at the interface between subunits. Can bind around 80 Ca(2+) ions. Regulates the release of lumenal Ca(2+) via the calcium release channel RYR1; this plays an important role in triggering muscle contraction. Negatively regulates store-operated Ca(2+) entry (SOCE) activity.

References:

[1]. Emiliano J Sanchez, et al. High-capacity Ca2+ binding of human skeletal calsequestrin. J Biol Chem. 2012 Mar 30;287(14):11592-601. [Content Brief]

[2]. Lane Zhang, et al. Calsequestrin-1 Regulates Store-Operated Ca2+ Entry by Inhibiting STIM1 Aggregation. Cell Physiol Biochem. 2016;38(6):2183-93. [Content Brief]

[3]. Virginia Barone, et al. Identification and characterization of three novel mutations in the CASQ1 gene in four patients with tubular aggregate myopathy. Hum Mutat. 2017 Dec;38(12):1761-1773. [Content Brief]

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