Very high magnification micrograph showing ragged red fibres (also ragged red fibers), commonly abbreviated RRF, in a mitochondrial myopathy. Gomori trichrome stain.
Mitochondrial Myopathies
Mitochondrial Myopathies
- Maternally-inherited
- Ragged red fibers / Subsarcolemmal accumulation of abnormal mitochondria
- Mitochondrial diseases result from abnormalities in mitochondria, which are key to energy production. Cell death occurs in the setting of significant failure. Ultimately this leads to damage in certain organ systems in particular: brain, heart, liver, skeletal muscles, kidney and the endocrine and respiratory systems.
- Symptoms may include loss of motor control, muscle weakness and pain, gastro-intestinal disorders and swallowing difficulties, poor growth, cardiac disease, liver disease, diabetes, respiratory complications, seizures, visual/hearing problems, lactic acidosis, developmental delays and susceptibility to infection."