Aberrant development of neuromuscular junctions in glycosylation-defective Largemyd mice
作者： Ruth Herbst , Thomas Iskratsch , Ewald Unger , Reginald E. Bittner
DOI: 10.1016/J.NMD.2009.02.011
关键词: Agrin 、 Myogenesis 、 Neuromuscular junction 、 Dystroglycan 、 Acetylcholine receptor 、 Anatomy 、 Neuromuscular junction disease 、 Biology 、 Skeletal muscle 、 Postsynaptic potential 、 Cell biology 、 Genetics(clinical) 、 Pediatrics, Perinatology, and Child Health 、 Neurology 、 Clinical neurology
摘要: Mice deficient in the glycosyltransferase Large are characterized by severe muscle and central nervous system abnormalities. In this study, we show that formation maintenance of neuromuscular junctions Largemyd mice greatly compromised. Neuromuscular not confined to endplate zone but widely spread frequently accompanied exuberant nerve sprouting. Nerve terminals highly fragmented binding α-bungarotoxin postsynaptic acetylcholine receptors (AChRs) is reduced. vitro, myotubes responsive agrin produce aberrant AChR clusters, which larger area less densely packed with AChRs. addition, expression on cell surface diminished suggesting assembly or transport defective. These results together finding O-linked glycosylation at compromised indicate action necessary for proper junction development.
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sci-hub.se 参考文章(70)
Mariko Taniguchi, Hiroki Kurahashi, Satoru Noguchi, Takayasu Fukudome, Takeshi Okinaga, Toshifumi Tsukahara, Youichi Tajima, Keiichi Ozono, Ichizo Nishino, Ikuya Nonaka, Tatsushi Toda, Aberrant neuromuscular junctions and delayed terminal muscle fiber maturation in α-dystroglycanopathies Human Molecular Genetics. ,vol. 15, pp. 1279- 1289 ,(2006) , 10.1093/HMG/DDL045
T. Misgeld, T. T. Kummer, J. W. Lichtman, J. R. Sanes, Agrin promotes synaptic differentiation by counteracting an inhibitory effect of neurotransmitter. Proceedings of the National Academy of Sciences of the United States of America. ,vol. 102, pp. 11088- 11093 ,(2005) , 10.1073/PNAS.0504806102
M. Peyrard, E. Seroussi, A.-C. Sandberg-Nordqvist, Y.-G. Xie, F.-Y. Han, I. Fransson, J. Collins, I. Dunham, M. Kost-Alimova, S. Imreh, J. P. Dumanski, THE HUMAN LARGE GENE FROM 22Q12.3-Q13.1 IS A NEW, DISTINCT MEMBER OF THE GLYCOSYLTRANSFERASE GENE FAMILY Proceedings of the National Academy of Sciences of the United States of America. ,vol. 96, pp. 598- 603 ,(1999) , 10.1073/PNAS.96.2.598
R Kornfeld, S E Tollefsen, The B4 lectin from Vicia villosa seeds interacts with N-acetylgalactosamine residues alpha-linked to serine or threonine residues in cell surface glycoproteins. Journal of Biological Chemistry. ,vol. 258, pp. 5172- 5176 ,(1983) , 10.1016/S0021-9258(18)32554-7
D Blanchard, C Capon, Y Leroy, J P Cartron, B Fournet, Comparative study of glycophorin A derived O-glycans from human Cad, Sd(a+) and Sd(a-) erythrocytes. Biochemical Journal. ,vol. 232, pp. 813- 818 ,(1985) , 10.1042/BJ2320813
S E Tollefsen, R Kornfeld, Isolation and characterization of lectins from Vicia villosa. Two distinct carbohydrate binding activities are present in seed extracts. Journal of Biological Chemistry. ,vol. 258, pp. 5165- 5171 ,(1983) , 10.1016/S0021-9258(18)32553-5
A. S. Ryder-Cook, P. Sicinski, K. Thomas, K. E. Davies, R. G. Worton, E. A. Barnard, M. G. Darlison, P. J. Barnard, Localization of the mdx mutation within the mouse dystrophin gene. The EMBO Journal. ,vol. 7, pp. 3017- 3021 ,(1988) , 10.1002/J.1460-2075.1988.TB03165.X
X. Zhu, C. Lai, S. Thomas, S. J. Burden, Neuregulin receptors, erbB3 and erbB4, are localized at neuromuscular synapses. The EMBO Journal. ,vol. 14, pp. 5842- 5848 ,(1995) , 10.1002/J.1460-2075.1995.TB00272.X
P N Kao, A J Dwork, R R Kaldany, M L Silver, J Wideman, S Stein, A Karlin, Identification of the alpha subunit half-cystine specifically labeled by an affinity reagent for the acetylcholine receptor binding site. Journal of Biological Chemistry. ,vol. 259, pp. 11662- 11665 ,(1984) , 10.1016/S0021-9258(20)71257-3
Patrice D. Côté, Hakima Moukhles, Michael Lindenbaum, Salvatore Carbonetto, Chimaeric mice deficient in dystroglycans develop muscular dystrophy and have disrupted myoneural synapses. Nature Genetics. ,vol. 23, pp. 338- 342 ,(1999) , 10.1038/15519