Dictionary of Human Evolution and Biology

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Mitochondrial Myopathies

Group of inherited disorders that cause profound muscle weakness and that are inherited strictly from the mother (maternal inheritance). The mitochondrial myopathic disorders include KSS (Kearns-Sayre syndrome), LHON (Leber hereditary optic neuropathy), infantile bilateral striatal necrosis, NARP (neuropathy, ataxia, and retinitis pigmentosa), MILS (maternally inherited Leigh syndrome), MELAS (mitochondrial encephalopathy lacticacidosis syndrome), PEO (progressive external ophthalmoplagia), and MERRF (myoclonic epilepsy with red ragged fibers: see epilepsy).

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