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1 β GalT IgA SNP IgA IgA IgA IgA O IgA IgA IgA IgA IgA O IgA N β GalT-I KO IgA IgA IgA IgA β GalT-I KO IgA K., & Mestecky, J.J. Clin. Invest.,, Miyawaki, S., Muso, E., Takeuchi, E., Matsushima, H., Shibata, Y., Sasayama, S., & Yoshida, H.Nephron,, Zheng, F., Kundu, G.C., Zhang, Z., Ward, J., DeMayo, F., & Mukherjee, A.B.Nat. Med.,, Launay, P., Grossetete, B., Arcos-Fajardo, M., Gaudin, E., Torres, S.P., Beaudoin, L., Patey-Mariaud de Serre, N., Lehuen, A.,&Monteiro,R.C.J. Exp. Med.,, Wang, J., Anders, R.A., Wu, Q., Peng, D., Cho, J.H., Sun, Y., Karaliukas, R., Kang, H.S., Turner, J.R., & Fu, Y.X.J. Clin. Invest.,, Asano, M., Furukawa, K., Kido, M., Matsumoto, S., Umesaki, Y., Kochibe, N., & Iwakura, Y.EMBO J.,, Asano, M., Nakae, S., Kotani, N., Shirafuji, N., Nambu, A., Hashimoto, N., Kawashima, H., Hirose, M., Miyasaka, M., Takasaki, S., & Iwakura, Y.Blood,, Mori, R., Kondo, T., Nishie, T., Ohshima, T., & Asano, M. Am. J. Pathol.,, Lu, Q., Hasty, P., & Shur, B.D.Dev. Biol.,,. Nishie, T., Miyaishi, O., Azuma, H., Kameyama, A., Naruse, C., Hashimoto, N., Yokoyama, H., Narimatsu, H., Wada, T., & Asano, M.Am. J. Pathol., Horie, A., Hiki, Y., Odani, H., Yasuda, Y., Takahashi, M., Kato, M., Iwase, H., Kobayashi, Y., Nakashima, I., & Maeda, K.Am.J.KidneyDis.,, Aberrant glycosylation of IgA and IgA nephropathy Masahide Asano and Toshikazu Nishie Division of Transgenic Animal Science, Kanazawa University Advanced Science Research Center, Takara-machi, Kanazawa, Japan Odani, H., Hiki, Y., Takahashi, M., Nishimoto, A., Yasuda, Y., Iwase, H., Shinzato, T., & Maeda, K.Biochem. Biophys. Res. Commun.,, Allen, A.C., Bailey, E.M., Brenchley, P.E., Buck, K.S., Barratt, J., & Feehally, J.Kidney Int.,, Kokubo, T., Hiki, Y., Iwase, H., Tanaka, A., Toma, K., Hotta, K., & Kobayashi, Y.J. Am. Soc. Nephrol.,, Sano, T., Hiki, Y., Kokubo, T., Iwase, H., Shigematsu, H., & Kobayashi, Y.Nephrol. Dial. Transplant.,, Tomana, M., Novak, J., Julian, B.A., Matousovic, K., Konecny, GM

2 A Hex B SD β- Hex GM GM GM HEXA HEXB GM A Hex α β GM Hex α β αβ HexA GM GM α β GM GM HEXA HEXB GM A Tay-Sachs Sandhoff AB variant A Hex β Hexb KO Sandhoff SD GM B SD Sandhoff SD SD GM GM

3 A SD MIP- α mrna B SD MIP- α C Sandhoff Wada SD CD Mac- α tumor necrosis factor α TNFα Jeyakumar MHC inos SD macrophage inflammatory protein- α MIP- α SD A B MIP- α GMGlcNAc MIP- α Wu SD

4 MIP- α GM SD Norflus SD GM X-GlcNAc Hex Jeyakumar N -butyldeoxynojirimycin NB-DNJ SD NB-DNJ GM GA Jeyakumar SD A Hex β B SD C SD

5 Jeyakumar L- SD SD Bourgoin SD A B Cachon-Gonzalez Hex α β protein transduction domain PTD SD Sandhoff GM GM Gravel, R.A., Kaback, M.M., Proia, R.L., Sandhoff, K., & Suzuki, K.The Metabolic and Molecular Bases of Inherited Disease, th ed. Scriver,C.R.,Beaudet,A.L.,Sly,W. S.,&Valle,D.ed., pp., McGraw-Hill, New York. Sango, K., Yamanaka, S., Hoffmann, A., Okuda, Y., Grinberg, A., Westphal, H., McDonald, M.P., Crawley, J.N., Sandhoff, K., Suzuki, K., & Proia, R.L.Nat. Genet.,,. Wada, R., Tifft, C.J., & Proia, R.L.Proc. Natl. Acad. Sci. USA,, Jeyakumar, M., Thomas, R., Elliot-Smith, E., Smith, D.A., van der Spoel., A.C., d Azzo, A., Perry, V.H., Butters, T.D., Dwek, R.A., & Platt, F.M.Brain,, Tsuji, D., Kuroki, A., Ishibashi, Y., Itakura, T., Kuwahara, J., Yamanaka, S., & Itoh, K.J. Neurochem.,, Wu,Y.P.&Proia,R.L.Proc. Natl. Acad. Sci. USA,, Norflus, F., Tifft, C.J., McDonald, M.P., Glodstein, G., Crawley, J.N., Hoffmann, A., Sandhoff, K., Suzuki, K., & Proia, R.L.J. Clin. Invest.,, Jeyakumar, M., Butters, T.D., Cortina-Borja, M., Hunnam, V., Proia, R.L., Perry, V.H., Dwek. R.A., & Platt, F.M. Proc. Natl. Acad. Sci. USA,, Jeyakumar, M., Norflus, F., Tifft, C.J., Cortina-Borja, M., Butters, T.D., Proia, R.L., Perry, V.H., Dwek, R.A., & Platt, F.M. Blood,, Jeyakumar, M., Smith, D.A., Willims, I.M., Borja, M.C., Neville, D.C.A., Butters, T.D., Dwek, R.A., & Platt, F.M. Ann. Neurol.,, Bourgoin, C., Emiliani, C., Kremer, E.J., Gelot, A., Tancini, B., Gravel, R.A., Drugan, C., Orlacchio, A., Poenaru, L., & Caillaud, C.Gene Ther.,, Tsuji, D., Kuroki, A., Ishibashi, Y., Itakura, T., & Itoh, K. J. Neurochem.,, Cachon-Gonzalez, M.B., Wang, S.Z., Lynch, A., Ziegler, R., Cheng, S.H., & Cox, T.M.Proc. Natl. Acad. Sci. USA,, Molecular pathogenesis and therapeutic targets of lysosomal diseases Daisuke Tsuji and Kohji Itoh Department of Medicinal Biotechnology, Institute of Health Biosciences, The University of Tokushima, Sho-machi, Tokushima, Japan L- γ- Pseudomonas putida L- γ- EC B γ- L-

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