Myosin, a hexamer of 2 identical heavy chains and 2 pairs of light chains, produces contractile force by moving along actin filaments and hydrolyzing ATP (1). The two pairs of light chains of muscle myosins are called essential light chains (ELC) and regulatory light chains (RLC). They stabilize the long alpha helical neck of the myosin head (2). Myosin light chain 2 (MYL2), also known as myosin II regulatory light chain, is a motor protein involved in the regulation of myosin ATPase activity in muscle cells (3). Calcium triggers the phosphorylation of MYL2 that in turn triggers myocardial contraction. Defects in MYL2 are the cause of cardiomyopathy familial hypertrophic type 10 (CMH10), a hereditary heart disorder characterized by ventricular hypertrophy (4).
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