title: Copper-metallothionein in the kidney of macular mice: a model for Menkes disease.
abstract: Menkes disease is an X-linked disorder of copper metabolism. Excess amounts of copper in the kidney of Macular mice, a model for this disease, were found as copper-metallothionein (Cu-MT) from kidney of the mice. Histochemical studies of Cu-MT based on its autofluorescent emission properties showed that the protein was predominant in the proximal convoluted tubule (PCT) cells of the cortex. PCT cells are known to be the primary site of the nephrotoxicity caused by heavy metals. MT mRNA was also observed in the cortex, indicating that the protein was biosynthesized in this region. On the basis of these results, we suggest that biosynthesis and degradation of Cu-MT occur repeatedly in the PCT cells of the cortex. We also compared the histochemical localization of Cu-MT in Macular mice and Long-Evans cinnamon rats, a model for Wilson's disease. The significance of this comparison is discussed.
authors.affiliation: Department of Environmental Medicine and Informatics, Graduate School of Environmental Earth Science, Hokkaido University, Sapporo, Japan.
authors.forename: M
authors.initials: M
authors.lastname: Suzuki-Kurasaki
authors.forename: M
authors.initials: M
authors.lastname: Okabe
authors.forename: M
authors.initials: M
authors.lastname: Kurasaki
mesh_terms: D000135:Acid Phosphatase / Q000032:analysis; D000818:Animals; D002352:Carrier Proteins / Q000737:chemistry / Q000302:isolation & purification; D003300:Copper / Q000008:administration & dosage; D004195:Disease Models, Animal; D006651:Histocytochemistry; D007279:Injections, Subcutaneous; D007668:Kidney / Q000737:chemistry* / Q000378:metabolism; D007672:Kidney Cortex / Q000737:chemistry; D007687:Kidney Tubules, Proximal / Q000737:chemistry / Q000648:ultrastructure; D008247:Lysosomes / Q000737:chemistry; D008297:Male; D007706:Menkes Kinky Hair Syndrome / Q000188:drug therapy / Q000378:metabolism* / Q000401:mortality; D008668:Metallothionein / Q000032:analysis* / Q000378:metabolism; D051379:Mice; D008809:Mice, Inbred C3H; D008815:Mice, Inbred Strains; D012333:RNA, Messenger / Q000032:analysis; D051381:Rats
chemical_list: D002352:Carrier Proteins; D012333:RNA, Messenger; C007121:copper thionein; C023548:copper-binding protein; D003300:Copper; D008668:Metallothionein; D000135:Acid Phosphatase