Mitochondrial gene expression and biogenesis in the cell dynamics

Mitochondria and their biological roles. Structure and functions of mitochondrial membranes. Inner mitochondrial membrane composition and permeability, electric potential properties. General aspects of transport processes across cell membranes. Mitochondrial fusion and fission. Mitophagy. Thermodynamic principles of chemical equilibrium, and their usefulness in defining some properties for non-equilibrium biological systems. Redox processes and their thermodynamic relationships. How and why ATP acts as energy currency in the cell. Energy charge and the adenylate pool.Redox processes and coenzymes in oxidative phosphorylation (OXPHOS). The mitochondrial electron transport chain (ETC) and its general mechanisms. The proton-motive force (Δp) generated by the ETC. The potential measurements in mitochondrial systems: the use of fluorescent probes to estimate the proton-motive force (Δp) from the IMM electrical potential.The electrons move between redox centres by the tunnel effect: the example of cytochrome c oxidase (COX). Redox reactions with the quinones/quinols in Complex II and III. How the Q cycle works. Reactive oxygen species (ROS) generation in mitochondria. The COX catalytic cycle: how proton pumping generates proton-motive force. The V complex or ATP synthase: rotational catalysis and some models for coupling proton transfer and ATP synthesis. The genomes in dual-membrane organelles. Evolutionary models of mitochondrial DNA (mtDNA). Mammalian and human mtDNA. Mitochondrial inheritance. mtDNA packing in the nucleoid. mtDNA and maternal inheritance. A plausible mechanism to prevent exceptions to an almost inflexible rule. The mitochondrial bottleneck in mammalian oogenesis. mtDNA mutation: a complex issue. mtDNA replication intermediates can be visualised by 2D-AGE. Recent advances on mtDNA/nuDNA interactions. Mitochondrial proteome expression. Strand L and H transcription from the HSP and LSP promoters. Cleavage and processing of primary transcripts from the H and L strands: many tRNAs “punctuate” the ORF in primary transcripts. Mitochondrial translation machinery: an overview of the translation cycle in a human mitoribosome. Import of cytoplasm-synthetized mitochondrial proteins.The architecture of mitochondrial compartments and its organizing complexes. Coordinate expression of mitochondrial proteins:how the expression of the bigenomic system is regulated. How nuclear genes coding for mitochondrial proteins are regulated in the nucleus. The topologically and topographically ordered assembly process for multi-subunit protein OXPHOS complexes: the example from Complex I; an almost complete assembly pathway of Complex I. Super-complexes, the respirasome and their possible functional significance. Genome editing technologies to mitochondria: some perspectives for gene therapy of diseases caused by mtDNA mutations using ZFN and TALEN. New developments for utilizing CRISPR–Cas9 and –Cas12a systems in mitochondrial genome editing: some challenges for localizing CRISPR components into mitochondria and for nuclease cleavage specificity. Local mitochondrial biogenesis in elongated neuronal cells, away from the cell soma. Anterograde and retrograde transport of mitochondria in neuronal extensions. mtDNA replication, mitochondrial fusion and fission in axons of peripheral neurons, different organelles move RNA granules. mRNA for nuclear-encoded mitochondrial proteins reach the neuron distal compartments to support the mitochondrial biogenesis processes in the neuron distal segments. In neuronal axons or dendrites, RNA granules can move with lysosome by a tethering annexin.Some specific issues on mitochondrial and cellular energetics. When chemical energy is transformed into heat: the brown adipose tissue and its mechanisms. Reverse electron transport and Δp generation through ATP hydrolysis: some noteworthy exceptions. The Inhibitor Protein hinders oligomycin activity on mitochondrial ATP synthase. Oligomycin Null-Point Test. A simplified functional approach to the quantitative evaluation of ATP phosphorylation potential in vivo. Mitochondria and aging: an ever-growing issue in mitochondrial biology.