线粒体通透性转变孔道与神经系统疾病

Mitochondrial permeability transition pore and neurologic disease

线粒体通透性转变孔道被认为可能主要由位于线粒体外膜的电压依赖性阴离子通道(voltage—dependent anion channel,VDAC)、位于内膜的腺苷酸转移酶(adenine nucleotide translocase,ANT)和位于线粒体基质的亲环素D(cyclophilin-D,CyP—D)组成。多种因素可以调节该孔道的形成和开放,包括小分子物质、信号传导通路以及蛋白与蛋白的相互作用。线粒体通透性转变孔道开放将增加线粒体内膜的通透性,导致线粒体功能紊乱,这一变化可能参与了神经系统疾病的病理发展过程。

Mitochondrial permeability transition pore （MPTP） is speculated to be composed of voltage- dependent anion channel （VDAC）, adenine nucleotide translocator （ANT）, and cyclophilin-D CYP-D. Many factors including small molecules, signaling kinase pathways, and protein-protein interaction could modulate opening of this pore. The opening of the pore could increase the permeability of mitochondrial inner membranes to ions and solutes thereby results in mitochondrial dysfunction, which may be involved in the pathology of neurologic diseases.