Merck
  • 主页
  • 查找结果
  • Restoration of copper metabolism and rescue of hepatic abnormalities in LEC rats, an animal model of Wilson disease, by expression of human ATP7B gene.

Restoration of copper metabolism and rescue of hepatic abnormalities in LEC rats, an animal model of Wilson disease, by expression of human ATP7B gene.

Biochimica et biophysica acta (2004-10-30)
Yan Meng, Ichiro Miyoshi, Masumi Hirabayashi, Mu Su, Yasumasa Mototani, Tadashi Okamura, Kunihiko Terada, Masatsugu Ueda, Katsuhiko Enomoto, Toshihiro Sugiyama, Noriyuki Kasai
摘要

Hepatic abnormalities in Long-Evans Cinnamon (LEC) rats, an animal model of Wilson disease (WD), were restored by the expression of the human ATP7B cDNA under the control of CAG promoter. Expression of ATP7B transcript and protein in the liver of the transgenic rats resulted in the restoration of biosynthesis of holoceruloplasmin and biliary copper excretion. Meanwhile, transgenic rats showed striking improvements in their hepatic abnormalities, i.e., rescue from fulminant hepatitis, late onset of hepatic cholangiofibrosis, suppression of hepatocellular carcinoma and much improved survival rates. Moreover, dramatic decreases were noted both in the levels of hepatic copper and iron in transgenic rats before the occurrence of hepatitis. These results indicated that the human ATP7B product compensated for the deficiency of the endogenous rattus protein and did function in intrahepatic copper transport by secreting copper into the plasma via incorporation into ceruloplasmin and by the excretion of copper into the bile, and that ATP7B is critical to hepatic dysfunctions in WD. This first successful transgenic rescue has important implications for the gene therapy of WD.

材料
货号
品牌
产品描述

Sigma-Aldrich
铜, powder, <425 μm, 99.5% trace metals basis
Sigma-Aldrich
铜, wire, diam. 1.0 mm, ≥99.9%
Sigma-Aldrich
铜, foil, thickness 0.25 mm, 99.98% trace metals basis
Sigma-Aldrich
铜, powder, 99.999% trace metals basis
Sigma-Aldrich
铜, nanopowder, 60-80 nm particle size (SAXS), ≥99.5% trace metals basis
Sigma-Aldrich
铜, ACS reagent, granular, 10-40 mesh, ≥99.90%
Sigma-Aldrich
铜, nanopowder, 40-60 nm particle size (SAXS), ≥99.5% trace metals basis
Sigma-Aldrich
铜, foil, ≥99.8% (complexometric)
Sigma-Aldrich
铜, foil, thickness 0.025 mm, 99.98% trace metals basis
Sigma-Aldrich
铜, powder, <75 μm, 99%
Sigma-Aldrich
铜, powder (spheroidal), 10-25 μm, 98%
Sigma-Aldrich
铜, turnings, purum p.a., ≥99.0%
Sigma-Aldrich
铜, foil, thickness 1.0 mm, 99.999% trace metals basis
Sigma-Aldrich
铜, powder (dendritic), <45 μm, 99.7% trace metals basis
Sigma-Aldrich
铜, wire, diam. 0.25 mm, 99.999% trace metals basis
Sigma-Aldrich
铜, foil, thickness 0.5 mm, 99.98% trace metals basis
Sigma-Aldrich
铜, spherical shot, −20-+30 mesh, ≥99.5% trace metals basis
Sigma-Aldrich
铜, bars (random sizes), ≥99.999% trace metals basis
Sigma-Aldrich
铜, wire, diam. 0.64 mm, 99.995% trace metals basis
Sigma-Aldrich
铜, electrolytic, Analytical Reagent, reag. Ph. Eur., ≥99.9% (complexometric), wire
Sigma-Aldrich
铜, rod, diam. 11 mm, 99.9999% trace metals basis
Sigma-Aldrich
铜, beads, 2-8 mm, ≥99.99% trace metals basis
Sigma-Aldrich
铜, beads, 2-8 mm, 99.9995% trace metals basis
Sigma-Aldrich
铜, shot, −3-+14 mesh, 99%
Sigma-Aldrich
铜, sputtering target, diam. × thickness 2.00 in. × 0.25 in., 99.95% trace metals basis
Sigma-Aldrich
铜, wire, diam. 2.0 mm, 99.999% trace metals basis
Supelco
铜(杂质), manufactured by MBH Analytical Ltd (39X 17868)
Sigma-Aldrich
铜, rod, diam. 3.1 mm, 99.999%
铜, IRMM®, certified reference material, 0.5 mm wire
铜, IRMM®, certified reference material, 0.1 mm foil