突触带是突触前蛋白复合物,其被认为对于视觉系统中的感觉信息的传递是重要的。丝带选择性地与那些突触相关联,其中膜电位的分级变化驱动持续的神经递质释放。缺陷的突触传递可能是单个带状成分诱变的结果。由于视网膜中带状突触的突触前分子机制的功能障碍而引起的视觉疾病很少见。在这次审查中,我们提供了引起视网膜功能障碍的突触病理学的概述,以及我们目前对其发病机制的理解,并讨论了在病理学中表现出带状突触参与的肌营养不良。
Synaptic ribbons are presynaptic protein complexes that are believed to be important for the transmission of sensory information in the visual system. Ribbons are selectively associated with those synapses where graded changes in membrane potential drive continuous neurotransmitter release. Defective synaptic transmission can arise as a result of the mutagenesis of a single ribbon component. Visual diseases that stem from malfunctions in the presynaptic molecular machinery of ribbon synapses in the retina are rare. In this
review, we provide an overview of synaptopathies that give rise to retinal malfunction and our present understanding of the mechanisms that underlie their pathogenesis and discuss muscular dystrophies that exhibit ribbon synapse involvement in the pathology.