Reduced proliferative activity of primary POMGnT1-null myoblasts in vitro

被引:32
作者
Miyagoe-Suzuki, Yuko [1 ]
Masubuchi, Nami [1 ,2 ]
Miyamoto, Kaori [1 ,2 ]
Wada, Michiko R. [1 ]
Yuasa, Shigeki [3 ]
Saito, Fumiaki [4 ]
Matsumura, Kiichiro [4 ]
Kanesaki, Hironori [5 ]
Kudo, Akira [5 ]
Manya, Hiroshi [6 ]
Endo, Tamao [6 ]
Takeda, Shin'ichi [1 ]
机构
[1] Natl Ctr Neurol & Psychiat, Natl Inst Neurosci, Dept Mol Therapy, Tokyo 1878502, Japan
[2] Kitasato Univ, Sch Sci, Dept Biosci, Kanagawa 2288555, Japan
[3] Natl Ctr Neurol & Psychiat, Natl Inst Neurosci, Dept Ultrastruct Res, Tokyo 1878502, Japan
[4] Teikyo Univ, Sch Med, Dept Neurol & Neurosci, Itabashi Ku, Tokyo 1738605, Japan
[5] Tokyo Inst Technol, Dept Biol Informat, Yokohama, Kanagawa 2268501, Japan
[6] Fdn Res Aging & Promot Human Welf, Tokyo Metropolitan Inst Gerontol, Glycobiol Res Grp, Itabashi Ku, Tokyo 1730015, Japan
关键词
POMGnT1; Muscle-eye-brain disease; Satellite cells; Skeletal muscle; alpha-Dystroglycan; Glycosylation; Laminin; DYSTROPHIN-ASSOCIATED GLYCOPROTEINS; CONGENITAL MUSCULAR-DYSTROPHIES; EYE-BRAIN DISEASE; SKELETAL-MUSCLE; SATELLITE CELLS; DYSTROGLYCAN; HYPERTROPHY; DISRUPTION; EXPRESSION; MICE;
D O I
10.1016/j.mod.2008.12.001
中图分类号
Q [生物科学];
学科分类号
07 ; 0710 ; 09 ;
摘要
Protein O-linked mannose beta 1,2-N-acetylglucosaminyltransferase 1 (POMGnT1) is an enzyme that transfers N-acetylglucosamine to O-mannose of glycoproteins. Mutations of the POMGnT1 gene cause muscle-eye-brain (MEB) disease. To obtain a better understanding of the pathogenesis of MEB disease, we mutated the POMGnT1 gene in mice using a targeting technique. The mutant muscle showed aberrant glycosylation of alpha-DG, and alpha-DG from mutant muscle failed to bind laminin in a binding assay. POMGnT1(-/-) muscle showed minimal pathological changes with very low-serum creatine kinase levels, and had normally formed muscle basal lamina, but showed reduced muscle mass, reduced numbers of muscle fibers, and impaired muscle regeneration. Importantly, POMGnT1(-/-) satellite cells proliferated slowly, but efficiently differentiated into multinuclear myotubes in vitro. Transfer of a retrovirus vector-mediated POMGnT1 gene into POMGnT1(-/-) myoblasts completely restored the glycosylation of alpha-DG, but proliferation of the cells was not improved. Our results suggest that proper glycosylation of alpha-DG is important for maintenance of the proliferative activity of satellite cells in vivo. (C) 2008 Elsevier Ireland Ltd. All rights reserved.
引用
收藏
页码:107 / 116
页数:10
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