Mitochondrion: Its Genetic Implications

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Journal of Genetic Syndromes & Gene Therapy has NLM ID: 101574143: Index Copernicus Value 2016: 84.15 and Impact Factor 2.34*

The mitochondrion (plural mitochondria) is a double membrane-bound organelle found in most eukaryotic organisms. Some cells in some multicellular organisms lack mitochondria (for example, mature mammalian red blood cells). A number of unicellular organisms, such as microsporidia, parabasalids, and diplomonads, have reduced or transformed their mitochondria into other structures. To date, only one eukaryote, Monocercomonoides, is known to have completely lost its mitochondria, and one multicellular organism, Henneguya salminicola, is known to have retained mitochondrion-related organelles in association with a complete loss of their mitochondrial genome. Mitochondria are an essential cell organelle for all living cells, if they are eliminated from the cell that means the cell has undergone terminal differentiation. It works like sub-kingdom in the cell; in addition to provide energy to the cell it has its own gasket of synthetic machinery. In each individual it is contributed by mother. If there is any defect (mitochondrial DNA mutation) in this organelle whole body suffers in a variety of ways. Recent researches are contributed towards corrections of mitochondrial defects by mitochondrial replacement or by infusion of healthy mitochondria.

Mitochondria are commonly between 0.75 and 3 μm² in area but vary considerably in size and structure. Unless specifically stained, they are not visible. In addition to supplying cellular energy, mitochondria are involved in other tasks, such as signalling, cellular differentiation, and cell death, as well as maintaining control of the cell cycle and cell growth. Mitochondrial biogenesis is in turn temporally coordinated with these cellular processes. Mitochondria have been implicated in several human diseases and conditions, such as mitochondrial disorders, cardiac dysfunction,  heart failure  and autism.

Using histochemical techniques for the first time Ward presented evidence for the origin of crystals within a single crista of mitochondria In some chondrocytes, several crystals were found, sometimes combined in a single mitochondrion. Crystals were preferentially aligned along the long axis of the cells, thus appearing in the same orientation as the chondrocytes in the tissue . Later, we have shown DNA in mitochondria and hence protein synthesis can take place within the mitochondria. Out of 37 genes thirteen provide instructions for making enzymes involved in oxidative phosphorylation. However for the first time we have shown that succinic dehydrogenase, the marker enzyme, is synthesized under nuclear as well mitochondrial genome.

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Christiane Zweier

Associate Editor

Journal of Genetic Syndromes & Gene Therapy