Calsequestrin-2

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, especially at the interface between subunits. Can bind around 60 Ca(2+) ions. Regulates the release of lumenal Ca(2+) via the calcium release channel RYR2; this plays an important role in triggering muscle contraction. Plays a role in excitation-contraction coupling in the heart and in regulating the rate of heart beats.

References:

[1]. Eunjung Kim, et al. Characterization of human cardiac calsequestrin and its deleterious mutants. J Mol Biol. 2007 Nov 2;373(4):1047-57. [Content Brief]

[2]. Emiliano J Sanchez, et al. Phosphorylation of human calsequestrin: implications for calcium regulation. Mol Cell Biochem. 2011 Jul;353(1-2):195-204. [Content Brief]

[3]. Giorgia Valle, et al. Catecholaminergic polymorphic ventricular tachycardia-related mutations R33Q and L167H alter calcium sensitivity of human cardiac calsequestrin. Biochem J. 2008 Jul 15;413(2):291-303. [Content Brief]

[4]. Marina Raffaele di Barletta, et al. Clinical phenotype and functional characterization of CASQ2 mutations associated with catecholaminergic polymorphic ventricular tachycardia. Circulation. 2006 Sep 5;114(10):1012-9. [Content Brief]

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