Abnormal Cellular Copper Metabolism in the Blotchy Mouse
| Autor: | C. H. Hill, Barry C. Starcher, David Fisk, Judy A. Madaras, Elizabeth F. Perry |
|---|---|
| Rok vydání: | 1978 |
| Předmět: |
Male
Mutant Medicine (miscellaneous) chemistry.chemical_element Locus (genetics) Electron Transport Complex IV Protein-Lysine 6-Oxidase Mice Animals Cytochrome c oxidase Tissue Distribution Allele Alleles Cells Cultured Metal Metabolism Inborn Errors Nutrition and Dietetics biology Molecular mass Ceruloplasmin Proteins Metabolism Fibroblasts Molecular biology Copper Molecular Weight Zinc chemistry Mutation biology.protein |
| Zdroj: | The Journal of Nutrition. 108:1229-1233 |
| ISSN: | 0022-3166 |
| DOI: | 10.1093/jn/108.8.1229 |
| Popis: | 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. |
| Databáze: | OpenAIRE |
| Externí odkaz: |
|