Literature DB >> 6290431

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

H C Liu, W Y Chan, O M Rennert.   

Abstract

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.

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Year:  1982        PMID: 6290431     DOI: 10.1007/bf01033627

Source DB:  PubMed          Journal:  Histochem J        ISSN: 0018-2214


  8 in total

1.  Cytochemical demonstration of succinic dehydrogenase by the use of a new p-nitrophenyl substituted ditetrazole.

Authors:  M M NACHLAS; K C TSOU; E DE SOUZA; C S CHENG; A M SELIGMAN
Journal:  J Histochem Cytochem       Date:  1957-07       Impact factor: 2.479

2.  Histochemical studies on the lateral geniculate bodies and visual cortices after optic nerve ligation in the mouse.

Authors:  D T Yew; H C Liu
Journal:  Acta Anat (Basel)       Date:  1981

3.  Cell culture studies of Menkes kinky hair disease.

Authors:  W Y Chan; A D Garnica; O M Rennert
Journal:  Clin Chim Acta       Date:  1978-09-15       Impact factor: 3.786

Review 4.  Prenatal and postnatal diagnosis of diseases of copper metabolism.

Authors:  W Y Chan; O M Rennert
Journal:  Ann Clin Lab Sci       Date:  1982 Sep-Oct       Impact factor: 1.256

5.  Genetic expression of Wilson's disease in cell culture: a diagnostic marker.

Authors:  W Y Chan; W Cushing; M A Coffman; O M Rennert
Journal:  Science       Date:  1980-04-18       Impact factor: 47.728

6.  Ceruloplasmin: the copper transport protein with essential oxidase activity.

Authors:  E Frieden; H S Hsieh
Journal:  Adv Enzymol Relat Areas Mol Biol       Date:  1976

7.  The role of copper in iron metabolism.

Authors:  W Y Chan; O M Rennert
Journal:  Ann Clin Lab Sci       Date:  1980 Jul-Aug       Impact factor: 1.256

8.  Inducibility of metallothionein biosynthesis in cultured normal and Menkes kinky hair disease fibroblasts: effects of copper and cadmium.

Authors:  W Y Chan; A D Garnica; O M Rennert
Journal:  Pediatr Res       Date:  1979-03       Impact factor: 3.756

  8 in total
  3 in total

1.  Effect of medium copper concentration on the growth, uptake and intracellular balance of copper and zinc in Menkes' and normal control cells.

Authors:  M H Rayner; K T Suzuki
Journal:  Biometals       Date:  1994-07       Impact factor: 2.949

2.  Succinate dehydrogenase activity in cultured human skin fibroblasts and amniotic fluid cells. A methodological study.

Authors:  T L Hansen; H Andersen
Journal:  Histochemistry       Date:  1983

3.  Decreased activity of cytochrome c oxidase in the macular mottled mouse: an immuno-electron microscopic study.

Authors:  K Seki; T Sato; Y Ishigaki; S Nakamura; Y Ishihara; T Ozawa
Journal:  Acta Neuropathol       Date:  1989       Impact factor: 17.088

  3 in total

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