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Mitochondrial myopathies are types of myopathies associated with mitochondrial disease.[1] On biopsy, the muscle tissue of patients with these diseases usually demonstrate "ragged red" muscle fibers. These ragged-red fibers contain mild accumulations of glycogen and neutral lipids, and may show an increased reactivity for succinate dehydrogenase and a decreased reactivity for cytochrome c oxidase. Inheritance was believed to be maternal (non-Mendelian extranuclear). It is now known that certain nuclear DNA deletions can also cause mitochondrial myopathy such as the OPA1 gene deletion. There are several subcategories of mitochondrial myopathies.

Mitochondrial myopathy
Animal mitochondrion diagram en (edit).svg
Simplified structure of a typical mitochondrion
Classification and external resources
Specialty neurology
ICD-10 G71.3
MeSH D017240
Orphanet 206966


Signs and symptomsEdit

Signs and symptoms include (for each of the following causes):



Gomori thrichrome stain: ragged red fibers


Although no cure currently exists, there is hope in treatment for this class of hereditary diseases with the use of an embryonic mitochondrial transplant.[2]


  1. ^ "Mitochondrial Myopathy Information Page | National Institute of Neurological Disorders and Stroke". Retrieved 2017-02-28. 
  2. ^ "Three-parent embryo formed in lab" (web). Scientists believe they have made a potential breakthrough in the treatment of serious disease by creating a human embryo with three separate parents. BBC News. 5 February 2008. Retrieved 2008-02-08. 

External linksEdit