Skip to main content
PMC home page
Biochemical Journal logoLink to Biochemical Journal
. 1982 Feb 15;202(2):369–371. doi: 10.1042/bj2020369

Copper metabolism in mottled mouse mutants. The effect of copper therapy on lysyl oxidase activity in brindled (Mobr) mice.

P M Royce, J Camakaris, J R Mann, D M Danks
PMCID: PMC1158120  PMID: 6124241

Abstract

Lysyl oxidase activity in extracts of skin from 1-day-old Mobr/Y mice was found to be between 50 and 60% of that in corresponding extracts from littermate +/Y mice of the same age. It was increased to 84-150% of that in the latter by prior treatment of the Mobr/Y mice at 7 days of age with a single subcutaneous injection of 50 micrograms of copper, retained as Cu+ in an alkyl polyether/sebacic acid solution. This suggests that in this form the copper is able to by-pass the block in copper metabolism and is deliverable to copper-requiring processes.

Full text

PDF
371

Selected References

These references are in PubMed. This may not be the complete list of references from this article.

  1. Camakaris J., Danks D. M., Ackland L., Cartwright E., Borger P., Cotton R. G. Altered copper metabolism in cultured cells from human Menkes' syndrome and mottled mouse mutants. Biochem Genet. 1980 Feb;18(1-2):117–131. doi: 10.1007/BF00504364. [DOI] [PubMed] [Google Scholar]
  2. Camakaris J., Mann J. R., Danks D. M. Copper metabolism in mottled mouse mutants: copper concentrations in tissues during development. Biochem J. 1979 Jun 15;180(3):597–604. doi: 10.1042/bj1800597. [DOI] [PMC free article] [PubMed] [Google Scholar]
  3. Daish P., Wheeler E. M., Roberts P. F., Jones R. D. Menkes's syndrome. Report of a patient treated from 21 days of age with parenteral copper. Arch Dis Child. 1978 Dec;53(12):956–958. doi: 10.1136/adc.53.12.956. [DOI] [PMC free article] [PubMed] [Google Scholar]
  4. Evans G. W., Reis B. L. Impaired copper homeostasis in neonatal male and adult female Brindled (Mobr) mice. J Nutr. 1978 Apr;108(4):554–560. doi: 10.1093/jn/108.4.554. [DOI] [PubMed] [Google Scholar]
  5. Garnica A. D., Frias J. L., Rennert O. M. Menkes kinky hair syndrome: Is it a treatable disorder? Clin Genet. 1977 Feb;11(2):154–161. doi: 10.1111/j.1399-0004.1977.tb01294.x. [DOI] [PubMed] [Google Scholar]
  6. Goka T. J., Stevenson R. E., Hefferan P. M., Howell R. R. Menkes disease: a biochemical abnormality in cultured human fibroblasts. Proc Natl Acad Sci U S A. 1976 Feb;73(2):604–606. doi: 10.1073/pnas.73.2.604. [DOI] [PMC free article] [PubMed] [Google Scholar]
  7. Hunt D. M. Primary defect in copper transport underlies mottled mutants in the mouse. Nature. 1974 Jun 28;249(460):852–854. doi: 10.1038/249852a0. [DOI] [PubMed] [Google Scholar]
  8. Mann J. R., Camakaris J., Danks D. M. Copper metabolism in mottled mouse mutants. Defective placental transfer of 64Cu to foetal brindled (Mobr) mice. Biochem J. 1980 Feb 15;186(2):629–631. doi: 10.1042/bj1860629. [DOI] [PMC free article] [PubMed] [Google Scholar]
  9. Mann J. R., Camakaris J., Danks D. M. Copper metabolism in mottled mouse mutants: distribution of 64Cu in brindled (Mobr) mice. Biochem J. 1979 Jun 15;180(3):613–619. doi: 10.1042/bj1800613. [DOI] [PMC free article] [PubMed] [Google Scholar]
  10. Mann J. R., Camakaris J., Danks D. M., Walliczek E. G. Copper metabolism in mottled mouse mutants: copper therapy of brindled (Mobr) mice. Biochem J. 1979 Jun 15;180(3):605–612. doi: 10.1042/bj1800605. [DOI] [PMC free article] [PubMed] [Google Scholar]
  11. Rayton J. K., Harris E. D. Induction of lysyl oxidase with copper. Properties of an in vitro system. J Biol Chem. 1979 Feb 10;254(3):621–626. [PubMed] [Google Scholar]
  12. Rowe D. W., McGoodwin E. B., Martin G. R., Grahn D. Decreased lysyl oxidase activity in the aneurysm-prone, mottled mouse. J Biol Chem. 1977 Feb 10;252(3):939–942. [PubMed] [Google Scholar]
  13. Rowe D. W., McGoodwin E. B., Martin G. R., Sussman M. D., Grahn D., Faris B., Franzblau C. A sex-linked defect in the cross-linking of collagen and elastin associated with the mottled locus in mice. J Exp Med. 1974 Jan 1;139(1):180–192. doi: 10.1084/jem.139.1.180. [DOI] [PMC free article] [PubMed] [Google Scholar]
  14. Royce P. M., Camakaris J., Danks D. M. Reduced lysyl oxidase activity in skin fibroblasts from patients with Menkes' syndrome. Biochem J. 1980 Nov 15;192(2):579–586. doi: 10.1042/bj1920579. [DOI] [PMC free article] [PubMed] [Google Scholar]
  15. Sayed A. K., Edwards J. A., Bannerman R. M. Copper metabolism of cultured fibroblasts from the brindled mouse (gene symbol Mobr). Proc Soc Exp Biol Med. 1981 Jan;166(1):153–156. doi: 10.3181/00379727-166-41038. [DOI] [PubMed] [Google Scholar]
  16. Siegel R. C. Lysyl oxidase. Int Rev Connect Tissue Res. 1979;8:73–118. doi: 10.1016/b978-0-12-363708-6.50009-6. [DOI] [PubMed] [Google Scholar]

Articles from Biochemical Journal are provided here courtesy of The Biochemical Society

RESOURCES