Mitochondrial ion channels as therapeutic targets

Abstract 

The study of mitochondrial ion channels changed our perception of these double-wrapped organelles from being just the power house of a cell to the guardian of a cell’s fate. Mitochondria communicate with the cell through these special channels. Most of the time, the message is encoded by ion flow across the mitochondrial outer and inner membranes. Potassium, sodium, calcium, protons, nucleotides, and proteins traverse the mitochondrial membranes in an exquisitely regulated manner to control a myriad of processes, from respiration and mitochondrial morphology to cell proliferation and cell death. This review is an update on both well established and putative mitochondrial channels regarding their composition, function, regulation, and therapeutic potential.

Abbreviations: AAA, alkaline-induced anion-selective activity, ACA, alkaline-induced cation-selective activity, ANT, adenosine nucleotide translocator, CLIC, chloride intracellular channel, IMAC, inner membrane anion channel, MAC, mitochondrial apoptosis-induced channel, mCS, mitochondrial Centum-pico-Siemen channel, MCU, mitochondrial calcium uniporter, PTP, permeability transition pore, TIM, translocase of the inner membrane, TOM, translocase of the outer membrane, VDAC, voltage dependent anion-selective channel

Keywords: Mitochondrial ion channels, Patch clamp, Mitochondrial disease, Therapy