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The mitochondrial genetic code is and example of a .

Mitochondria are responsible for creating more than 90% of the energy needed by the body to sustain life and support organ function. When they fail, less and less energy is generated within the cell. Cell injury and even cell death follow. If this process is repeated throughout the body, whole organ systems begin to fail.

T1 - ATP synthesis is coupled to rat liver mitochondrial RNA synthesis

In eukaryotic cells, glycolysis and fermentation reactions occur in the cytoplasm. The remaining pathways, starting with pyruvate oxidation, occur in the mitochondria. Most eukaryotic mitochondria can use only oxygen as the terminal electron acceptor for respiration. In the presence of oxygen, pyruvate enters the mitochondrial matrix and is oxidized to acetyl-CoA, and then to CO2 via the citric acid cycle. The electron transport chain and ATP synthase are located on the mitochondrial inner membrane.

ATP Synthase Animation - Sumanas, Inc.

Fox TD (2012) Mitochondrial protein synthesis, import, and assembly. Genetics 192 (4): 1203–1234.

The inner membrane has infoldings called the cristae that increase the surface area for the complexes and proteins that aid in the production of ATP, the energy rich molecules.

Mitochondrial genes in animals include those for a few of the proteins needed for oxidative phosphorylation (the rest are encoded by nuclear genes)(), mitochondrial rRNA genes, and most of the mitochondrial tRNA genes ().

ATP Synthase | MRC Mitochondrial Biology Unit

The enzyme (also in the same membrane) that catalyzed production ATP reaction is ATP synthase.

Further evidence to support the endosymbiont theory is that mitochondria have their own DNA, in the form of a circular chromosome that is topologically like bacterial chromosomes. The sequence of the mitochondrial DNA most closely resembles the sequences of genes in alpha-proteobacteria. Mitochondrial ribosomes are structurally more similar to bacterial ribosomes than to eukaryotic ribosomes. Mitochondria reproduce in eukaryotic cells by fission, again resembling bacterial cell division.

Moraes CT, Diaz F and Barrientos A (2004) Defects in the biosynthesis of mitochondrial heme c and heme a in yeast and mammals. Biochimica et Biophysica Acta 1659 (2‐3): 153–159.

In order for this synthesis to work, it is necessary to move ATP out of, and ADP into the matrix.
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(substrates for ATP synthesis) into the mitochondria.

Though the primary function of mitochondria is to produce energy, they also play an important role in the metabolism and synthesis of certain other substances in the body.

ATP Synthase - The European Bioinformatics Institute

Rat liver mitochondria respond to changes in energy demand by modulating the amount of RNA synthesized. Coupled rat liver mitochondria were used to determine the relationship between mitochondrial respiration, ATP levels, and mitochondrial transcription. This system included oxidizable substrates (malate and glutamate) and constituents that could support both mitochondrial respiration and transcription. The respiratory inhibitor rotenone, phosphorylation inhibitor oligomycin, and the uncoupler of oxidative phosphorylation carbonyl-cyanide p-triflouromethoxyphehylhydrazone inhibited RNA synthesis. Addition of ADP stimulated mitochondrial transcription and peak RNA synthesis was observed at 1-2 mM ADP. At ADP concentrations above 2 mM, RNA synthesis decreased. These results demonstrate that mitochondrial transcription is tightly coupled to ATP levels.

How ATP is produced in both the chloroplast and mitochondria.

Allen JF (1993) Control of gene expression by redox potential and the requirement for chloroplast and mitochondrial genomes. Journal of Theoretical Biology 165 (4): 609–631.

Mitochondria-ATP - Pure Encapsulations

N2 - Rat liver mitochondria respond to changes in energy demand by modulating the amount of RNA synthesized. Coupled rat liver mitochondria were used to determine the relationship between mitochondrial respiration, ATP levels, and mitochondrial transcription. This system included oxidizable substrates (malate and glutamate) and constituents that could support both mitochondrial respiration and transcription. The respiratory inhibitor rotenone, phosphorylation inhibitor oligomycin, and the uncoupler of oxidative phosphorylation carbonyl-cyanide p-triflouromethoxyphehylhydrazone inhibited RNA synthesis. Addition of ADP stimulated mitochondrial transcription and peak RNA synthesis was observed at 1-2 mM ADP. At ADP concentrations above 2 mM, RNA synthesis decreased. These results demonstrate that mitochondrial transcription is tightly coupled to ATP levels.

In animals the site of Energy is Mitochondria

AB - Rat liver mitochondria respond to changes in energy demand by modulating the amount of RNA synthesized. Coupled rat liver mitochondria were used to determine the relationship between mitochondrial respiration, ATP levels, and mitochondrial transcription. This system included oxidizable substrates (malate and glutamate) and constituents that could support both mitochondrial respiration and transcription. The respiratory inhibitor rotenone, phosphorylation inhibitor oligomycin, and the uncoupler of oxidative phosphorylation carbonyl-cyanide p-triflouromethoxyphehylhydrazone inhibited RNA synthesis. Addition of ADP stimulated mitochondrial transcription and peak RNA synthesis was observed at 1-2 mM ADP. At ADP concentrations above 2 mM, RNA synthesis decreased. These results demonstrate that mitochondrial transcription is tightly coupled to ATP levels.

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