Cytochrome C oxidase deficiency and neuronal involvement in Menkes' kinky hair disease: immunohistochemical study.

@article{Sparaco1993CytochromeCO,
  title={Cytochrome C oxidase deficiency and neuronal involvement in Menkes' kinky hair disease: immunohistochemical study.},
  author={M Sparaco and Asao Hirano and M Hirano and Salvatore Dimauro and Eduardo Bonilla},
  journal={Brain pathology},
  year={1993},
  volume={3 4},
  pages={349-54}
}
Antibodies against subunits II and IV of cytochrome c oxidase (COX) and against complex III of the respiratory chain were used to study the expression of these proteins in the cerebellum, spinal cord, and other regions of the central nervous system in an autoptic case of Menkes' kinky hair disease (MKHD). We found a reduced expression of COX subunits in all examined areas whereas staining for complex III appeared normal. Immunostaining was altered in morphologically well-preserved neurons… CONTINUE READING
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