---blotchy red muscle fibers on Gomori trichrome stain
-abnormal mitochondria accumulate under sarcolemma of muscle fibers
-- most important types of the mitochondrial myopathies are:
1). Myoclonic epilepsy with ragged red fibers
2). Lever optic neuropathy
3). Mitochondrial encephalopathy with stroke-like episodes and lactic acidosis
These disease are caused by different impairments of mtDNA.
As we remember sperm mitochondria do not pass into the ovum during fertilization, but transmitted only maternal mitochondria to the mitochondria. ====>> these disease have maternal inheritance .
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