title: Metallothionein in kidney and liver of the macular mouse as an animal model of Menkes' kinky hair disease.
abstract: The tissue copper and metallothionein-Cu (MT-Cu) content of kidney and liver were measured in mutant (hemizygous macular male and homozygous macular female), heterozygous macular female and normal mouse. The tissue copper and MT-Cu contents in kidneys from 7-8 day mutants and heterozygotes were significantly greater than those of the normal kidney. Marked elevations in kidney copper and MT-Cu contents were also observed in the 8-9 week mutant (which achieved long-term survival with a single dose of subcutaneous copper administered at day 7) and in the heterozygote. The L-[35S]cystine incorporation experiments also revealed an abnormal synthesis of renal MT in the 8-9 week mutant and in the heterozygote. In contrast to kidney Cu levels, the tissue copper and MT-Cu contents of 7-8 day normal livers were extremely high, whereas the tissue copper and MT-Cu contents of mutant and heterozygote livers were extremely low. The tissue copper contents of livers of 8-9 week mutants and heterozygotes were slightly low compared to normal, and the MT-Cu contents of livers of the 8-9 week mouse were extremely low in all groups. In contrast to the changes in copper content, the changes in tissue zinc and MT-Zn contents in kidney and liver were slight in the 7-8 day and 8-9 week mouse.
authors.affiliation: Department of Radiation Medicine, Okayama University Medical School, Japan.
authors.forename: N
authors.initials: N
authors.lastname: Shiraishi
authors.forename: S
authors.initials: S
authors.lastname: Kondoh
authors.forename: Y
authors.initials: Y
authors.lastname: Hiraki
authors.forename: K
authors.initials: K
authors.lastname: Aono
authors.forename: T
authors.initials: T
authors.lastname: Taguchi
mesh_terms: D000818:Animals; D001928:Brain Diseases, Metabolic / Q000378:metabolism*; D003300:Copper / Q000378:metabolism*; D003600:Cytosol / Q000378:metabolism; D004195:Disease Models, Animal; D005260:Female; D006580:Genetic Carrier Screening; D007668:Kidney / Q000378:metabolism*; D008099:Liver / Q000378:metabolism*; D008297:Male; D007706:Menkes Kinky Hair Syndrome / Q000378:metabolism*; D008668:Metallothionein / Q000378:metabolism*; D051379:Mice; D008817:Mice, Mutant Strains; D013054:Spectrophotometry, Atomic; D015032:Zinc / Q000378:metabolism*
chemical_list: D003300:Copper; D008668:Metallothionein; D015032:Zinc