Mitochondrial encephalopathy, MELAS


An eye disease called Leber’s hereditary optic atrophy; and
A type of epilepsy called MERRF which stands for Myoclonus Epilepsy with Ragged Red Fibers.

The mitochondria, as mentioned, are normal structures located outside the nucleus in the cell’s cytoplasm. Each mitochondrion has a chromosome made of DNA that is quite different from the better known chromosomes in the nucleus. The mitochondrial chromosome is much smaller; it is round (whereas the chromosomes in the nucleus are normally shaped like rods); there are many copies of the mitochondrial chromosome in every cell; and no matter whether we are male or female, we inherit our mitochondrial chromosome from our mother.

MELAS and all other mitochondrial diseases were entirely enigmatic before it was discovered that they were due to mutations not in regular chromosomes but in the chromosome of mitochondria.

Read Also:

  • Mitochondrial genome

    The sum of the genetic information contained in the chromosome of the mitochondrion, a structure located in the cytoplasm outside the nucleus of the cell. The mitochondrial genome is composed of mitochondrial DNA (mDNA), a double-stranded circular molecule that contains a limited number of genes. During fertilization, mDNA is transmitted only by the mother. Together, […]

  • Mitochondrial inheritance

    //ghr.nlm.nih.gov/chromosome=MT>

  • Mitochondrial myopathy

    A group of neuromuscular diseases caused by damage to the mitochondria, energy-producing structures in cells that serve as power plants. Nerve and muscle cells require a great deal of energy and are particularly impaired by mitochondrial dysfunction. Some of the more common mitochondrial myopathies include the Kearns-Sayre syndrome, myoclonic epilepsy with ragged-red fibers, and the […]

  • Mitochondrion

    //ghr.nlm.nih.gov/chromosome=MT>

  • Mitosis

    The ordinary division of a body cell (a somatic cell) to form two daughter cells, each with the same chromosome complement as the parent cell.


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