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Junctional sarcoplasmic reticulum protein 1 (JSRP1), also known as JP-45, is an integral protein of the junctional face membrane of the skeletal muscle sarcoplasmic reticulum. It plays a critical role in skeletal muscle excitation-contraction coupling by interacting with the ryanodine receptor, calsequestrin, and the L-type voltage-dependent calcium channel Cav1.1 (CACNA1S)[1][2][3][4][5][6][7][8]. JSRP1 helps regulate calcium influx and efflux, modulating the function and membrane targeting of voltage-sensitive calcium channels and potentially influencing the phenotype of rare muscle disorders such as Brody disease and modifying susceptibility in malignant hyperthermia[6][8]. Mutations in JSRP1 can decrease the sensitivity of the dihydropyridine receptor (CACNA1S) to activation, affecting excitation-contraction coupling efficiency in skeletal muscle[4][6][8]. No direct drugs or known pharmacological modulators targeting JSRP1 are reported in the literature as of the current date.
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