Article
Cytochrome c oxidase-intermediate fibres: importance in understanding the pathogenesis and treatment of mitochondrial myopathy.
Neuromuscular disorders : NMD - 1 Aug 2012
Murphy Julie L, Ratnaike Thiloka E, Shang Ersong, Falkous Gavin, Blakely Emma L, Alston Charlotte L, Taivassalo Tanja, Haller Ronald G, Taylor Robert W, Turnbull Doug M
Abstract excerpt
An important diagnostic muscle biopsy finding in patients with mitochondrial DNA disease is the presence of respiratory-chain deficient fibres. These fibres are detected as cytochrome c oxidase-deficient following a sequential cytochrome c oxidase-succinate dehydrogenase reaction, often in a mosaic pattern within a population of cytochrome c oxidase-normal fibres. Detailed analysis of muscle biopsies from...
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