Altered copper metabolism in cultured cells from human Menkes' syndrome and mottled mouse mutants

@article{Camakaris1980AlteredCM,
  title={Altered copper metabolism in cultured cells from human Menkes' syndrome and mottled mouse mutants},
  author={James Camakaris and David M. Danks and Leigh Ackland and Elizabeth Cartwright and Pamela Borger and Richard G. H. Cotton},
  journal={Biochemical Genetics},
  year={1980},
  volume={18},
  pages={117-131}
}
Cultured cells of a variety of different types from human Menkes' syndrome patients and brindled mouse mutants exhibit similarly altered responses to changes in extracellular copper concentration. This suggests that the mutations in the mouse and human are very similar and that mutant gene expression is occurring in many different tissues. Intracellular copper levels are markedly elevated in mutant cells in normal medium and in medium containing a hundredfold higher copper. Some cell lines from… CONTINUE READING