Copper metabolism in the macular mutant mouse: An animal model of menkes’s kinky-hair disease

Noriyuki Shiraishi, K. Aono, Takehito Taguchi

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19 Citations (Scopus)


The tissue copper contents were measured in mutant (hemizygous macular male and homozygous macular female), heterozygous macular female and normal mice. The cop-per content in kidney and small intestine from 7-day-old mutant and heterozygote were extremely high compared to normal mice, whereas the copper content in other tissues (liver, brain, spleen and serum) was low. Copper content in whole body of mutant mice was extremely low at three stages (18 days gestation, 1 day old, and 7 days old) compared to normal mice with the exception of the mutant fetus at 14 days of gestation. Renal copper contents in the mutant fetus at 18 days of gestation and in the 1-day-old mutant were not changed compared to normal mice, whereas that in 7-and 14-day-old mutant mice was significantly elevated. Hepatic copper content of the mutant mice was extremely low at all stages compared to normal mice. Copper therapy was applied to 7-day-old mutant mice. At 1 day after injection, hepatic copper content in the mutants had increased slightly in comparison with the normal control mice, whereas renal copper content was extremely increased. At 7 days after injection, hepatic copper content in the mutants was decreased greatly in comparison with normal control mice, whereas an increase in renal copper content had remained.

Original languageEnglish
Pages (from-to)173-180
Number of pages8
Issue number3
Publication statusPublished - 1988
Externally publishedYes



  • Copper metabolism
  • Kidney
  • Liver
  • Macular mouse
  • Menkes’s kinky-hair disease

ASJC Scopus subject areas

  • Developmental Biology
  • Pediatrics, Perinatology, and Child Health

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