Muscular dystrophy with laminin deficiency decreases the content f butyrylcholinesterase tetramers in sciatic nerves of Lama2dy mice
Muscular dystrophy with laminin deficiency decreases the content f butyrylcholinesterase tetramers in sciatic nerves of Lama2dy mice
复制标题
DOI：
10.1016/s0304-3940(02)00885-6
复制
发表时间：
2002-10-18
影响因子：
2.5
通讯作者：
Campoy, FJ
中科院分区：
文献类型：
--
作者：
Moral-Naranjo, MT;Cabezas-Herrera, J;Campoy, FJ
Lama2dy小鼠是先天性肌营养不良症（DMD）的模型，表现为肌肉变性和周围神经的髓鞘异常。虽然有报道称MCMD可降低小鼠坐骨神经乙酰胆碱酯酶（AChE）活性，但其对丁基胆碱酯酶（BuChE）的作用尚未见报道。在小鼠坐骨神经中发现了两亲性BuChE单体(G（1)(A), 39%）、二聚体(G（2)(A), 18%）、四聚体(G（4)(A), 33%）以及亲水性四聚体(G（4)(H), 10%）。含有丰富的G(4)(A)（75%）和较少的G(1)(A)、G(2)(A)、G(4)(H)和A(12) AChE成分。在营养不良的神经中，BuChE活性增加了2倍，但G(4)(A)形式的比例从33%下降到10%。乙酰胆碱酯酶活性降低，酶的组成不受影响。凝集素相互作用研究表明，与骨骼肌相反，美罗红蛋白的缺陷并没有很大地改变神经胆碱酯酶的糖基化。营养不良神经中BuChE形态的异常合成可能与MCMD周围神经病变有关。(C) 2002年由爱思唯尔科学爱尔兰有限公司出版
The Lama2dy mouse, a model of congenital muscular dystrophy (DMD) by merosin deficiency (MCMD), shows muscle degeneration and dysmyelination of peripheral nerves. Although it has been reported that MCMD reduces acetylcholinesterase (AChE) activity of mouse sciatic nerve, no information is available regarding its action on butyrylcholinesterase (BuChE). Amphiphilic BuChE monomers (G(1)(A), 39%), dimers (G(2)(A), 18%), and tetramers (G(4)(A), 33%), along with hydrophilic tetramers (G(4)(H), 10%), were identified in mouse sciatic nerve. It also contained abundant G(4)(A) (75%) and less G(1)(A), G(2)(A), G(4)(H) and A(12) AChE components. In dystrophic nerves, the BuChE activity increased 2-fold but the proportion of the G(4)(A) form dropped from 33% to 10%. AChE activity decreased and the composition of enzyme forms was unaffected. Lectin interaction studies showed that, in contrast to skeletal muscle, the defect of merosin did not greatly alter the glycosylation of nerve cholinesterases. The anomalous synthesis of BuChE forms in dystrophic nerve may be related with peripheral neuropathy of MCMD. (C) 2002 Published by Elsevier Science Ireland Ltd.