Department of Molecular Neuroscience, UCL Institute of Neurology, London, UK.
Adv Exp Med Biol. 2013;961:241-9. doi: 10.1007/978-1-4614-4756-6_20.
Mitochondrial Na(+)/Ca(2+) exchange (NCXmito) is critical for neuronal Ca(2+) homeostasis and prevention of cell death from excessive mitochondrial Ca(2+) (m[Ca(2+)]) accumulation. The mitochondrial kinase PINK1 appears to regulate the mCa(2+) efflux from dopaminergic (DAergic) neurons, possibly by directly regulating NCXmito. However, the precise molecular identity of NCXmito is unknown and has been the subject of great controversy. Here we propose that the previously characterised plasmalemmal NCX isoforms (NCX2, NCX3) contribute to mitochondrial Na(+)/Ca(2+) exchange in human DAergic neurons and may act downstream of PINK1 in the prevention of neurodegeneration by m[Ca(2+)] accumulation. Firstly, we definitively show the existence of a mitochondrial pool of endogenous plasmalemmal NCX isoforms in human DAergic neurons and cell lines using immunolocalisation and fluorescence-assisted organelle sorting (FAOS). Secondly, we demonstrate reduced mitochondrial Ca(2+) efflux occurs following inhibition of NCX2 or NCX3 (but not NCX1) using siRNA or antibody blocking. This study has potentially revealed a new molecular target in Parkinson's disease pathology which ultimately may open up new avenues for future therapeutic intervention.
线粒体钠/钙交换(NCXmito)对于神经元钙稳态和防止因线粒体钙(m[Ca(2+))积累过多而导致的细胞死亡至关重要。线粒体激酶 PINK1 似乎可以调节多巴胺能(DAergic)神经元的 mCa(2+)外排,可能是通过直接调节 NCXmito。然而,NCXmito 的精确分子身份尚不清楚,一直是一个很大的争议。在这里,我们提出以前表征的质膜 NCX 同工型(NCX2、NCX3)有助于人类 DAergic 神经元中线粒体的钠/钙交换,并且可能在通过 m[Ca(2+)]积累预防神经退行性变中作用于 PINK1 的下游。首先,我们使用免疫定位和荧光辅助细胞器分选(FAOS)明确证明了人类 DAergic 神经元和细胞系中存在内源性质膜 NCX 同工型的线粒体池。其次,我们证明了使用 siRNA 或抗体阻断抑制 NCX2 或 NCX3(但不是 NCX1)后,线粒体 Ca(2+)的外排减少。这项研究可能揭示了帕金森病病理中的一个新的分子靶点,最终可能为未来的治疗干预开辟新的途径。
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