Calsequestrin-2 (dCASQ2) Dog Native Protein

calsequestrin 2: Calsequestrin is a high-capacity, moderate affinity, calcium-binding protein and thus acts as an internal calcium store in muscle. The release of calcium bound to calsequestrin through a calcium release channel triggers muscle contraction. The skeletal muscle isoform (CASQ1) binds around 80 Ca(2+) ions, while the cardiac isoform (CASQ2) binds approximately 60 Ca(2+) ions. Defects in CASQ2 are the cause of catecholaminergic polymorphic ventricular tachycardia type 2 (CPVT2); also known as stress-induced polymorphic ventricular tachycardia (VTSIP). CPVT2 is an autosomal recessive form of arrhythmogenic disorder characterized by stress-induced, bidirectional ventricular tachycardia that may degenerate into cardiac arrest and cause sudden death. Belongs to the calsequestrin family.

Protein type: Calcium-binding

Cellular Component: sarcoplasmic reticulum lumen; sarcoplasmic reticulum; junctional membrane complex; voltage-gated calcium channel complex; Z disc

Molecular Function: protein binding; calcium ion binding; calcium-dependent protein binding

Biological Process: protein polymerization; regulation of heart rate; sequestering of calcium ion; sarcomere organization; cardiac muscle contraction