D. Etiology Mitochondrial Cytopathy

1138 Words Feb 18th, 2018 5 Pages
Mitochondrial cytopathy is a genetic heritable disorder [5]. It occurs as a result of DNA mutation in the gem-line cells that can be transmitted to the second generation. This type of genetic disorder is often caused by mutations in the mitochondrial DMA versus the nucleic DNA. The mitochondria DNA is 20-30 times more susceptible to acquire mutations secondary to absence of DNA repair mechanisms in the mitochondria, giving rise to frequent point mutations or deletions in the mtDNA during cell division[1]. Such mutations are inherited exclusively from the maternal mitochondria. The paternal mitochondria do not contribute to the fetal mitochondria [6]. When some of the mitochondria in the ovum have mutations in their DNA, some of those defected mitochondria will go to daughter cells upon division. If the cells receiving the defected mtDNA contribute to forming tissues that are actively dividing after birth, they will be eliminated by the natural selection process after successive cell divisions. In contrast, if the cells inheriting the defected mitochondria developed into organs or tissues of limited dividing ability, this will result in problems related to energy metabolism in that organ [6]. Due to the random nature of the process, defected mitochondria may end up randomly in different types of tissue and at different concentration. This explains the variations in the manifestation, progression, prognosis and severity of the disorder.
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