Cytochrome c oxidase and coenzyme Q in neuromuscular diseases: a histochemical study

C. Doriguzzi, L. Palmucci, B. Pollo, T. Mongini, M. Maniscalco, L. Chiadò-Piat, D. Schiffer

Research output: Contribution to journalArticlepeer-review


Cytochrome c oxidase (CCO) has been histochemically studied in 250 muscle biopsies from patients with different neuromuscular diseases. The results were compared with those obtained on serial sections stained with Gomori's trichrome and with the methods for NADH tetrazolium reductase, succinate dehydrogenase and lactate dehydrogenase. In 58 selected cases serial sections were also stained with a method demonstrating coenzyme Q (CoQ) activity. Demonstration of structural alterations was as good with CCO as with the methods for other oxidative enzymes: particularly evident were alterations of the distribution of mitochondria, such as core areas in central core and multiminicore diseases. Unstained fibers were observed in mitochondrial myopathies, in Becker, Emery-Dreifuss, limb-girdle, facio-scapulo-humeral muscular dystrophies, muscle infarction, polymyositis, motor neuron diseases and neuropathies. The histochemical method for CoQ showed only low specificity, since partial staining was also present in areas devoid of mitochondria, such as cores. CoQ deficiency was not observed in any of the 19 mitochondrial myopathies examined.

Original languageEnglish
Pages (from-to)25-29
Number of pages5
JournalActa Neuropathologica
Issue number1
Publication statusPublished - Jan 1990


  • Coenzyme Q
  • Cytochrome c oxidase
  • Hystochemistry
  • Muscle biopsy

ASJC Scopus subject areas

  • Neuroscience(all)
  • Pathology and Forensic Medicine
  • Clinical Neurology


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