Department of Biological Sciences, University of Pittsburgh, Pittsburgh, PA 15260, USA.
Cell Calcium. 2010 Feb;47(2):103-11. doi: 10.1016/j.ceca.2009.12.007. Epub 2010 Jan 6.
Lysosomal storage diseases (LSDs) are caused by inability of cells to process the material captured during endocytosis. While they are essentially diseases of cellular "indigestion", LSDs affect large number of cellular activities and, as such, they teach us about the integrative function of the cell, as well as about the gaps in our knowledge of the endocytic pathway and membrane transport. The present review summarizes recent findings on Ca2+ handling in LSDs and attempts to identify the key questions on alterations in Ca2+ signaling and membrane transport in this group of diseases, answers to which may lie in delineating the cellular pathogeneses of LSDs.
溶酶体贮积症(LSD)是由于细胞无法对内吞作用中捕获的物质进行加工而引起的。虽然它们本质上是细胞“消化不良”的疾病,但 LSD 会影响许多细胞活动,因此,它们让我们了解了细胞的整体功能,以及我们对胞吞途径和膜转运的认识中的空白。本综述总结了 LSD 中 Ca2+处理的最新发现,并试图确定该组疾病中 Ca2+信号和膜转运改变的关键问题,这些问题的答案可能在于阐明 LSD 的细胞发病机制。