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Abnormal cellular copper metabolism in the blotchy mouse.

Authors
  • Starcher, B
  • Madaras, J A
  • Fisk, D
  • Perry, E F
  • Hill, C H
Type
Published Article
Journal
The Journal of nutrition
Publication Date
Aug 01, 1978
Volume
108
Issue
8
Pages
1229–1233
Identifiers
PMID: 27591
Source
Medline
License
Unknown

Abstract

Defective copper metabolism was demonstrated in male mice bearing the blotchy (Moblo/y) allele at the mottled locus on the X-chromosome. Copper absorption from the gut was only 64% of that found in normal mice and hepatic copper levels were only 56% of the controls. Ceruloplasmin and heart cytochrome c oxidase activities were normal, yet lysyl oxidase activity from cultured fibroblasts was only 45% of control levels. Copper accumulated in fibroblasts cultured from these mutants to values that were five times normal. The accumulation of copper in the fibroblasts was associated with a protein of approximately 12,000 molecular weight.

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