Calcium misregulation and the pathogenesis of muscular dystrophy.

摘要: Although the exact nature of relationship between calcium and pathogenesis Duchenne muscular dystrophy (DMD) is not fully understood, this an important issue which has been addressed in several recent reviews (Alderton Steinhardt, 2000a, Gailly, 2002, Allen et al., 2005). A key question when trying to understand cellular basis DMD how absence or low level expression dystrophin, a cytoskeletal protein, results slow but progressive necrosis muscle fibres. loss sarcolemmal integrity from dystrophin clearly plays role associated with DMD, number lines evidence also establish for misregulation ions pathology, particularly cytoplasmic space just under sarcolemma. calcium-permeable channels have identified can exhibit greater activity dystrophic cells, existing suggests that these may represent different variants same channel type (perhaps transient receptor potential channel, TRPC). In addition, prominent calcium-activated proteases pathology established, as well modulation other intracellular regulatory proteins signaling pathways. Whether its direct regulation ions, stores, indirectly alters through enhancement membrane tearing, remains unclear. Here we focus on areas consensus divergence amongst literature, propose where future research would be especially valuable