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Increased globotriaosylceramide levels in a transgenic mouse expressing human 1,4-galactosyltransferase and a mouse model for treating Fabry disease

2010; Oxford University Press; Volume: 149; Issue: 2 Linguagem: Inglês

10.1093/jb/mvq125

1756-2651

Chikara SHIOZUKA, Ayumu Taguchi, Junichiro Matsuda, Yoko Noguchi, Takanori Kunieda, Kozue Uchio‐Yamada, Hidekatsu Yoshioka, Ryoji Hamanaka, S Yano, Shin‐ichiro Yokoyama, Kazuaki Mannen, Ashok B. Kulkarni, Koichi Furukawa, Shoken Ishii,

Carbohydrate Chemistry and Synthesis

Journal Article Increased globotriaosylceramide levels in a transgenic mouse expressing human α1,4-galactosyltransferase and a mouse model for treating Fabry disease Get access Chikara Shiozuka, Chikara Shiozuka 1Department of Agricultural and Life Sciences, Obihiro University of Agriculture and Veterinary Medicine, Obihiro, Hokkaido 080-8555; 2Department of Matrix Medicine, Faculty of Medicine, Oita University, Yufu, Oita 879-5593; 3Laboratory of Animal Models for Human Diseases, National Institute of Biomedical Innovation, Ibaraki, Osaka 567-0085; 4Department of Cell Biology, Faculty of Medicine, Oita University, Yufu, Oita 879-5593; 5Department of Diagnostic Pathology, Faculty of Medicine, Oita University, Yufu, Oita 879-5593; 6Division of Laboratory Animal Sciences, Research Promotion Project, Oita University, Yufu, Oita 879-5593, Japan; 7National Institute of Dental and Craniofacial Research, Bethesda, MD 20892, USA; 8Department of Biochemistry II, Nagoya University School of Medicine, Nagoya, Aichi 466-0065; and 9Biochemical Laboratory, GlycoPharma Corporation, Oita 870-0822, Japan Search for other works by this author on: Oxford Academic PubMed Google Scholar Atsumi Taguchi, Atsumi Taguchi 1Department of Agricultural and Life Sciences, Obihiro University of Agriculture and Veterinary Medicine, Obihiro, Hokkaido 080-8555; 2Department of Matrix Medicine, Faculty of Medicine, Oita University, Yufu, Oita 879-5593; 3Laboratory of Animal Models for Human Diseases, National Institute of Biomedical Innovation, Ibaraki, Osaka 567-0085; 4Department of Cell Biology, Faculty of Medicine, Oita University, Yufu, Oita 879-5593; 5Department of Diagnostic Pathology, Faculty of Medicine, Oita University, Yufu, Oita 879-5593; 6Division of Laboratory Animal Sciences, Research Promotion Project, Oita University, Yufu, Oita 879-5593, Japan; 7National Institute of Dental and Craniofacial Research, Bethesda, MD 20892, USA; 8Department of Biochemistry II, Nagoya University School of Medicine, Nagoya, Aichi 466-0065; and 9Biochemical Laboratory, GlycoPharma Corporation, Oita 870-0822, Japan Search for other works by this author on: Oxford Academic PubMed Google Scholar Junichiro Matsuda, Junichiro Matsuda 1Department of Agricultural and Life Sciences, Obihiro University of Agriculture and Veterinary Medicine, Obihiro, Hokkaido 080-8555; 2Department of Matrix Medicine, Faculty of Medicine, Oita University, Yufu, Oita 879-5593; 3Laboratory of Animal Models for Human Diseases, National Institute of Biomedical Innovation, Ibaraki, Osaka 567-0085; 4Department of Cell Biology, Faculty of Medicine, Oita University, Yufu, Oita 879-5593; 5Department of Diagnostic Pathology, Faculty of Medicine, Oita University, Yufu, Oita 879-5593; 6Division of Laboratory Animal Sciences, Research Promotion Project, Oita University, Yufu, Oita 879-5593, Japan; 7National Institute of Dental and Craniofacial Research, Bethesda, MD 20892, USA; 8Department of Biochemistry II, Nagoya University School of Medicine, Nagoya, Aichi 466-0065; and 9Biochemical Laboratory, GlycoPharma Corporation, Oita 870-0822, Japan Search for other works by this author on: Oxford Academic PubMed Google Scholar Yoko Noguchi, Yoko Noguchi 1Department of Agricultural and Life Sciences, Obihiro University of Agriculture and Veterinary Medicine, Obihiro, Hokkaido 080-8555; 2Department of Matrix Medicine, Faculty of Medicine, Oita University, Yufu, Oita 879-5593; 3Laboratory of Animal Models for Human Diseases, National Institute of Biomedical Innovation, Ibaraki, Osaka 567-0085; 4Department of Cell Biology, Faculty of Medicine, Oita University, Yufu, Oita 879-5593; 5Department of Diagnostic Pathology, Faculty of Medicine, Oita University, Yufu, Oita 879-5593; 6Division of Laboratory Animal Sciences, Research Promotion Project, Oita University, Yufu, Oita 879-5593, Japan; 7National Institute of Dental and Craniofacial Research, Bethesda, MD 20892, USA; 8Department of Biochemistry II, Nagoya University School of Medicine, Nagoya, Aichi 466-0065; and 9Biochemical Laboratory, GlycoPharma Corporation, Oita 870-0822, Japan Search for other works by this author on: Oxford Academic PubMed Google Scholar Takanori Kunieda, Takanori Kunieda 1Department of Agricultural and Life Sciences, Obihiro University of Agriculture and Veterinary Medicine, Obihiro, Hokkaido 080-8555; 2Department of Matrix Medicine, Faculty of Medicine, Oita University, Yufu, Oita 879-5593; 3Laboratory of Animal Models for Human Diseases, National Institute of Biomedical Innovation, Ibaraki, Osaka 567-0085; 4Department of Cell Biology, Faculty of Medicine, Oita University, Yufu, Oita 879-5593; 5Department of Diagnostic Pathology, Faculty of Medicine, Oita University, Yufu, Oita 879-5593; 6Division of Laboratory Animal Sciences, Research Promotion Project, Oita University, Yufu, Oita 879-5593, Japan; 7National Institute of Dental and Craniofacial Research, Bethesda, MD 20892, USA; 8Department of Biochemistry II, Nagoya University School of Medicine, Nagoya, Aichi 466-0065; and 9Biochemical Laboratory, GlycoPharma Corporation, Oita 870-0822, Japan Search for other works by this author on: Oxford Academic PubMed Google Scholar Kozue Uchio-Yamada, Kozue Uchio-Yamada 1Department of Agricultural and Life Sciences, Obihiro University of Agriculture and Veterinary Medicine, Obihiro, Hokkaido 080-8555; 2Department of Matrix Medicine, Faculty of Medicine, Oita University, Yufu, Oita 879-5593; 3Laboratory of Animal Models for Human Diseases, National Institute of Biomedical Innovation, Ibaraki, Osaka 567-0085; 4Department of Cell Biology, Faculty of Medicine, Oita University, Yufu, Oita 879-5593; 5Department of Diagnostic Pathology, Faculty of Medicine, Oita University, Yufu, Oita 879-5593; 6Division of Laboratory Animal Sciences, Research Promotion Project, Oita University, Yufu, Oita 879-5593, Japan; 7National Institute of Dental and Craniofacial Research, Bethesda, MD 20892, USA; 8Department of Biochemistry II, Nagoya University School of Medicine, Nagoya, Aichi 466-0065; and 9Biochemical Laboratory, GlycoPharma Corporation, Oita 870-0822, Japan Search for other works by this author on: Oxford Academic PubMed Google Scholar Hidekatsu Yoshioka, Hidekatsu Yoshioka 1Department of Agricultural and Life Sciences, Obihiro University of Agriculture and Veterinary Medicine, Obihiro, Hokkaido 080-8555; 2Department of Matrix Medicine, Faculty of Medicine, Oita University, Yufu, Oita 879-5593; 3Laboratory of Animal Models for Human Diseases, National Institute of Biomedical Innovation, Ibaraki, Osaka 567-0085; 4Department of Cell Biology, Faculty of Medicine, Oita University, Yufu, Oita 879-5593; 5Department of Diagnostic Pathology, Faculty of Medicine, Oita University, Yufu, Oita 879-5593; 6Division of Laboratory Animal Sciences, Research Promotion Project, Oita University, Yufu, Oita 879-5593, Japan; 7National Institute of Dental and Craniofacial Research, Bethesda, MD 20892, USA; 8Department of Biochemistry II, Nagoya University School of Medicine, Nagoya, Aichi 466-0065; and 9Biochemical Laboratory, GlycoPharma Corporation, Oita 870-0822, Japan Search for other works by this author on: Oxford Academic PubMed Google Scholar Ryoji Hamanaka, Ryoji Hamanaka 1Department of Agricultural and Life Sciences, Obihiro University of Agriculture and Veterinary Medicine, Obihiro, Hokkaido 080-8555; 2Department of Matrix Medicine, Faculty of Medicine, Oita University, Yufu, Oita 879-5593; 3Laboratory of Animal Models for Human Diseases, National Institute of Biomedical Innovation, Ibaraki, Osaka 567-0085; 4Department of Cell Biology, Faculty of Medicine, Oita University, Yufu, Oita 879-5593; 5Department of Diagnostic Pathology, Faculty of Medicine, Oita University, Yufu, Oita 879-5593; 6Division of Laboratory Animal Sciences, Research Promotion Project, Oita University, Yufu, Oita 879-5593, Japan; 7National Institute of Dental and Craniofacial Research, Bethesda, MD 20892, USA; 8Department of Biochemistry II, Nagoya University School of Medicine, Nagoya, Aichi 466-0065; and 9Biochemical Laboratory, GlycoPharma Corporation, Oita 870-0822, Japan Search for other works by this author on: Oxford Academic PubMed Google Scholar Shinji Yano, Shinji Yano 1Department of Agricultural and Life Sciences, Obihiro University of Agriculture and Veterinary Medicine, Obihiro, Hokkaido 080-8555; 2Department of Matrix Medicine, Faculty of Medicine, Oita University, Yufu, Oita 879-5593; 3Laboratory of Animal Models for Human Diseases, National Institute of Biomedical Innovation, Ibaraki, Osaka 567-0085; 4Department of Cell Biology, Faculty of Medicine, Oita University, Yufu, Oita 879-5593; 5Department of Diagnostic Pathology, Faculty of Medicine, Oita University, Yufu, Oita 879-5593; 6Division of Laboratory Animal Sciences, Research Promotion Project, Oita University, Yufu, Oita 879-5593, Japan; 7National Institute of Dental and Craniofacial Research, Bethesda, MD 20892, USA; 8Department of Biochemistry II, Nagoya University School of Medicine, Nagoya, Aichi 466-0065; and 9Biochemical Laboratory, GlycoPharma Corporation, Oita 870-0822, Japan Search for other works by this author on: Oxford Academic PubMed Google Scholar Shigeo Yokoyama, Shigeo Yokoyama 1Department of Agricultural and Life Sciences, Obihiro University of Agriculture and Veterinary Medicine, Obihiro, Hokkaido 080-8555; 2Department of Matrix Medicine, Faculty of Medicine, Oita University, Yufu, Oita 879-5593; 3Laboratory of Animal Models for Human Diseases, National Institute of Biomedical Innovation, Ibaraki, Osaka 567-0085; 4Department of Cell Biology, Faculty of Medicine, Oita University, Yufu, Oita 879-5593; 5Department of Diagnostic Pathology, Faculty of Medicine, Oita University, Yufu, Oita 879-5593; 6Division of Laboratory Animal Sciences, Research Promotion Project, Oita University, Yufu, Oita 879-5593, Japan; 7National Institute of Dental and Craniofacial Research, Bethesda, MD 20892, USA; 8Department of Biochemistry II, Nagoya University School of Medicine, Nagoya, Aichi 466-0065; and 9Biochemical Laboratory, GlycoPharma Corporation, Oita 870-0822, Japan Search for other works by this author on: Oxford Academic PubMed Google Scholar ... Show more Kazuaki Mannen, Kazuaki Mannen 1Department of Agricultural and Life Sciences, Obihiro University of Agriculture and Veterinary Medicine, Obihiro, Hokkaido 080-8555; 2Department of Matrix Medicine, Faculty of Medicine, Oita University, Yufu, Oita 879-5593; 3Laboratory of Animal Models for Human Diseases, National Institute of Biomedical Innovation, Ibaraki, Osaka 567-0085; 4Department of Cell Biology, Faculty of Medicine, Oita University, Yufu, Oita 879-5593; 5Department of Diagnostic Pathology, Faculty of Medicine, Oita University, Yufu, Oita 879-5593; 6Division of Laboratory Animal Sciences, Research Promotion Project, Oita University, Yufu, Oita 879-5593, Japan; 7National Institute of Dental and Craniofacial Research, Bethesda, MD 20892, USA; 8Department of Biochemistry II, Nagoya University School of Medicine, Nagoya, Aichi 466-0065; and 9Biochemical Laboratory, GlycoPharma Corporation, Oita 870-0822, Japan Search for other works by this author on: Oxford Academic PubMed Google Scholar Ashok B. Kulkarni, Ashok B. Kulkarni 1Department of Agricultural and Life Sciences, Obihiro University of Agriculture and Veterinary Medicine, Obihiro, Hokkaido 080-8555; 2Department of Matrix Medicine, Faculty of Medicine, Oita University, Yufu, Oita 879-5593; 3Laboratory of Animal Models for Human Diseases, National Institute of Biomedical Innovation, Ibaraki, Osaka 567-0085; 4Department of Cell Biology, Faculty of Medicine, Oita University, Yufu, Oita 879-5593; 5Department of Diagnostic Pathology, Faculty of Medicine, Oita University, Yufu, Oita 879-5593; 6Division of Laboratory Animal Sciences, Research Promotion Project, Oita University, Yufu, Oita 879-5593, Japan; 7National Institute of Dental and Craniofacial Research, Bethesda, MD 20892, USA; 8Department of Biochemistry II, Nagoya University School of Medicine, Nagoya, Aichi 466-0065; and 9Biochemical Laboratory, GlycoPharma Corporation, Oita 870-0822, Japan Search for other works by this author on: Oxford Academic PubMed Google Scholar Koichi Furukawa, Koichi Furukawa 1Department of Agricultural and Life Sciences, Obihiro University of Agriculture and Veterinary Medicine, Obihiro, Hokkaido 080-8555; 2Department of Matrix Medicine, Faculty of Medicine, Oita University, Yufu, Oita 879-5593; 3Laboratory of Animal Models for Human Diseases, National Institute of Biomedical Innovation, Ibaraki, Osaka 567-0085; 4Department of Cell Biology, Faculty of Medicine, Oita University, Yufu, Oita 879-5593; 5Department of Diagnostic Pathology, Faculty of Medicine, Oita University, Yufu, Oita 879-5593; 6Division of Laboratory Animal Sciences, Research Promotion Project, Oita University, Yufu, Oita 879-5593, Japan; 7National Institute of Dental and Craniofacial Research, Bethesda, MD 20892, USA; 8Department of Biochemistry II, Nagoya University School of Medicine, Nagoya, Aichi 466-0065; and 9Biochemical Laboratory, GlycoPharma Corporation, Oita 870-0822, Japan Search for other works by this author on: Oxford Academic PubMed Google Scholar Satoshi Ishii Satoshi Ishii * 1Department of Agricultural and Life Sciences, Obihiro University of Agriculture and Veterinary Medicine, Obihiro, Hokkaido 080-8555; 2Department of Matrix Medicine, Faculty of Medicine, Oita University, Yufu, Oita 879-5593; 3Laboratory of Animal Models for Human Diseases, National Institute of Biomedical Innovation, Ibaraki, Osaka 567-0085; 4Department of Cell Biology, Faculty of Medicine, Oita University, Yufu, Oita 879-5593; 5Department of Diagnostic Pathology, Faculty of Medicine, Oita University, Yufu, Oita 879-5593; 6Division of Laboratory Animal Sciences, Research Promotion Project, Oita University, Yufu, Oita 879-5593, Japan; 7National Institute of Dental and Craniofacial Research, Bethesda, MD 20892, USA; 8Department of Biochemistry II, Nagoya University School of Medicine, Nagoya, Aichi 466-0065; and 9Biochemical Laboratory, GlycoPharma Corporation, Oita 870-0822, Japan *Satoshi Ishii, Ph.D., Department of Matrix Medicine, Faculty of Medicine, Oita University, Hasama-cho Idaigaoka 1-1, Yufu-shi, Oita 879-5593, Japan, Tel: +81 97 586 5661, Fax: +81 97 546 2534, email: ishiis01@oita-u.ac.jp Search for other works by this author on: Oxford Academic PubMed Google Scholar The Journal of Biochemistry, Volume 149, Issue 2, February 2011, Pages 161–170, https://doi.org/10.1093/jb/mvq125 Published: 19 October 2010 Article history Received: 01 September 2010 Accepted: 14 October 2010 Published: 19 October 2010