Department of Biology, University of Padova, Via Ugo Bassi 58/B, 35131 Padova, Italy.
INFN, Department of Physics, University of Rome Tor Vergata, Via della Ricerca Scientifica 1, 00133 Rome, Italy.
Cells. 2022 Mar 8;11(6):921. doi: 10.3390/cells11060921.
A distinct set of channels and transporters regulates the ion fluxes across the lysosomal membrane. Malfunctioning of these transport proteins and the resulting ionic imbalance is involved in various human diseases, such as lysosomal storage disorders, cancer, as well as metabolic and neurodegenerative diseases. As a consequence, these proteins have stimulated strong interest for their suitability as possible drug targets. A detailed functional characterization of many lysosomal channels and transporters is lacking, mainly due to technical difficulties in applying the standard patch-clamp technique to these small intracellular compartments. In this review, we focus on current methods used to unravel the functional properties of lysosomal ion channels and transporters, stressing their advantages and disadvantages and evaluating their fields of applicability.
一套独特的通道和转运蛋白调节溶酶体膜的离子通量。这些转运蛋白的功能障碍以及由此导致的离子失衡与各种人类疾病有关，如溶酶体贮积症、癌症以及代谢和神经退行性疾病。因此，这些蛋白因其适合作为可能的药物靶点而引起了强烈的兴趣。许多溶酶体通道和转运蛋白的详细功能特征尚不清楚，主要是由于将标准膜片钳技术应用于这些小的细胞内隔室存在技术困难。在这篇综述中，我们重点介绍了用于揭示溶酶体离子通道和转运蛋白功能特性的当前方法，强调了它们的优缺点，并评估了它们的适用领域。
