Phenotypic Characterization of Ggt1dwg/dwg Mice,a Mouse Model for Hereditary γ-GlutamylTransferase Deficiency

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概要

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• Ggt1dwg/dwg mice are spontaneous mutant mice with a nucleotide deletion in the Ggt1 gene. They are characterized by dwarfism, cataract, and coat color abnormality. These abnormalities in the external appearance of Ggt1dwg/dwg mice closely resemble those of previously reported GGT1-deficient mice, Ggt1tm1Zuk/tm1Zuk (Ggt1-/-) and Ggt1enu1/enu1, generated by gene targeting or ENU mutagenesis. However, whether the pathological features of Ggt1dwg/dwg mice are also similar to those of the Ggt1-/- and Ggt1enu1/enu1 mice remains unclear. To clarify the pathogenesis of Ggt1dwg/dwg mice, we physiologically and histologically investigated the abnormalities of Ggt1dwg/dwg mice in this study. First, we analyzed the activity of GGT1 and GSH levels in Ggt1dwg/dwg mice. GGT1 activity in the Ggt1dwg/dwg mice was reduced to approximately 4.0% of that in the wild-type mice. Plasma and kidney GSH levels were markedly increased, while eye and liver GSH levels were markedly decreased, in the Ggt1dwg/dwg mice. Notably, no significant difference in survival rate was observed between the Ggt1dwg/dwg and wild-type mice, whereas high mortality was reported in the Ggt1-/- and Ggt1enu1/enu1 mice. Growth retardation, degeneration of lens fibers, and an increased number of osteoclasts in the Ggt1dwg/dwg mice were reversed by administration of N-acetyl-L-cysteine, a precursor of GSH synthesis. Thus, we conclude that the abnormalities of Ggt1dwg/dwg mice are caused by alteration of the GSH levels due to the depression of GGT1 activity and that Ggt1dwg/dwg mice will be a useful model for GGT deficiency with peculiar features.

著者

• Kunieda Tetsuo

  Graduate School Of Natural Sci. And Technol. Okayama Univ.

• Tsuji Takehito

  Graduate School Of Natural Science And Technology Okayama University

• YAMADA Kaoru

  Graduate School of Natural Science and Technology, Okayama University, 1-1-1 Tsushima-Naka, Kita-ku, Okayama 700-8530, Japan

• KUNIEDA Tetsuo

  Graduate School of Environmental and Life Science, Okayama University, 1-1-1 Tsushima-Naka, Kita-ku, Okayama 700-8530, Japan

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