The Histochemical Journal

, Volume 14, Issue 5, pp 781–789 | Cite as

Histochemical studies of fibroblasts from patients with Menkes kinky hair disease and Wilson's disease

  • H. C. Liu
  • W. Y. Chan
  • O. M. Rennert
Papers

Summary

Cultured fibroblasts from Menkes kinky hair disease patients showed markedly reduced succinate dehydrogenase and amine oxidase activities. Cytochrome oxidase activity, however, was greatly reduced in some cells and almost normal in others. Cultured fibroblasts from patients with Wilson's disease showed moderately reduced succinate dehydrogenase and cytochrome oxidase activities. Amine oxidase activity was only slightly reduced when compared to that of normal. These results indicated that the histochemical phenotype observed in fibroblasts from patients with Menkes kinky hair disease and Wilson's disease were distinctly different from each other and from normal fibroblasts.

Keywords

Succinate Disease Patient Oxidase Activity Cytochrome Oxidase Succinate Dehydrogenase 

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Copyright information

© Chapman and Hall Ltd. 1982

Authors and Affiliations

  • H. C. Liu
    • 1
  • W. Y. Chan
    • 2
  • O. M. Rennert
    • 2
  1. 1.Department of AnatomyUniversity of Hong KongHong Kong
  2. 2.Department of Pediatrics and Department of Biochemistry and Molecular BiologyUniversity of Oklahoma Health Sciences CenterOklahoma CityUSA

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