GD3-and O-acetylated GD3-gangliosides in the GM2 synthase-deficient mouse brain and their immunohistochemical localization

スポンサーリンク

概要

• 論文の詳細を見る

• Gangliosides in the brain of the knockout mouse deficient in the activity of β1,4 N-acetylgalactosaminyl transferase (β1,4 GalNAc-T)(GM2 synthase) consisted of nearly exclusively of GM3- and GD3-gangliosides as expected from the known substrate specificity of the enzyme and in confirmation of the initial reports from two laboratories that generated the mutant mouse experimentally. The total molar amount of gangliosides was approximately 30% higher in the mutant mouse brain than that in the wild-type brain. However, contrary to the initial reports, one-fourth of total GD3-ganglioside was O-acetylated. It reacted positively with an anti-O-acetylated GD3 monoclonal antibody and disappeared with a corresponding increase in GD3-ganglioside after mild alkaline treatment. The absence of O-acetylated GD3 in the initial reports can be explained by the saponification step included in their analytical procedures. Although quantitatively much less and identification tentative, we also detected GT3 and O-acetylated GT3. Anti-GD3 and anti-O-acetylated GD3 monoclonal antibodies gave positive reactions in the brain of mutant mouse as expected from the analytical results. Either antibody barely stained wild-type brain except for immunoreactivity of GD3 in the cerebellar Purkinje cells. The distributions of GD3 and O-acetylated GD3 in the brain of mutant mouse were similar but differential localization was noted in the cerebellar Purkinje cells and cerebral cortex. (Communicated by Tamio YAMAKAWA, M.J.A.)

著者

• VANIER Marie

  INSERM Unit 189, Lyon-Sud Medical School of Medicine

• Matsuda Junko

  Inst. Of Glycoscience Tokai Univ.

• Vanier Marie

  Inserm Unit 189 Lyon-sud Medical School Of Medicine

• Matsuda Junko

  Institute Of Glycoscience Tokai University

• SUZUKI Kunihiko

  Institute of Glycoscience, Tokai University

• POPA Iuliana

  EA-3732 University of Lyon-1, Edouard Herriot Hospital

• PORTOUKALIAN Jacques

  EA-3732 University of Lyon-1, Edouard Herriot Hospital

• SUZUKI Kunihiko

  Institute of Glycotechnology, Future Science and Technology Joint Research Center, Tokai University

• VANIER Marie

  INSERM U 499, RTH Laënnec School of Medicine, and Fondation Gillet-Mérieux, Lyon-Sud Hospital

関連論文

• Isolation of NPC1-Deficient Chinese Hamster Ovary Cell Mutants by Gene Trap Mutagenesis^1
• GD3-and O-acetylated GD3-gangliosides in the GM2 synthase-deficient mouse brain and their immunohistochemical localization
• Regional expression of prosaposin in the wild-type and saposin D-deficient mouse brain detected by an anti-mouse prosaposin-specific antibody
• Induced Mouse Models of Abnormal Sphingolipid Metabolism
• スフィンゴ糖脂質の脂肪酸C2位の水酸化は神経系において軸索およびミエリン鞘の長期的な維持に不可欠である
• Chemoenzymatic Synthesis of the Lipid Binding Protein, Saposin C
• Isolation of NPC 1-Deficient Chinese Hamster Ovary Cell Mutants by Gene Trap Mutagenesis.
• Improved High-Fat Diet-Induced Glucose Intolerance by an Oral Administration of Phytosphingosine

スポンサーリンク