The present invention relates to 1,4-benzothiazepine derivatives and their use to treat conditions, disorders and diseases associated with ryanodine receptors (RyRs) that regulate calcium channel functioning in cells. The invention also discloses pharmaceutical compositions comprising the compounds and uses thereof to treat diseases and conditions associated with RyRs, in particular cardiac, musculoskeletal and central nervous system (CNS) disorders.本發明係關於1,4-苯并噻氮呯衍生物及其治療與調控細胞中鈣通道功能之雷諾定受體(ryanodine receptor;RyR)相關病況、病症及疾病上之用途。本發明亦揭示含有該等化合物之醫藥組合物及其治療與RyR相關疾病及病況上之用途,特定言之是指心臟、肌肉骨骼及中樞神經系統(CNS)疾病。