DOWNS-SYNDROME - MORPHOLOGICAL REMODELING AND INCREASED COMPLEXITY IN THE NEUROMUSCULAR-JUNCTION OF TRANSGENIC CUZN-SUPEROXIDE DISMUTASE MICE
DOWNS-SYNDROME - MORPHOLOGICAL REMODELING AND INCREASED COMPLEXITY IN THE NEUROMUSCULAR-JUNCTION OF TRANSGENIC CUZN-SUPEROXIDE DISMUTASE MICE
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DOI：
10.1007/bf01186993
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发表时间：
1991-03-01
期刊：
影响因子：
--
通讯作者：
GRONER, Y
中科院分区：
文献类型：
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作者：
AVRAHAM, KB;SUGARMAN, H;GRONER, Y
利用携带人cuzn -超氧化物歧化酶基因的转基因小鼠，研究cuzn -超氧化物歧化酶基因剂量是否与唐氏综合征相关神经肌肉连接处恶化的迹象有关。研究了cuzn -超氧化物歧化酶转基因小鼠及其非转基因窝鼠后肢肌肉神经肌肉连接形态的三个参数：神经末梢长度、神经末梢分支点数量和导致突触形成的发芽发生率。这些参数随着年龄的增长而增加，并且在cuzn -超氧化物歧化酶转基因小鼠中增加的时间更早。因此，该数据符合cuzn -超氧化物歧化酶转基因小鼠在神经肌肉连接形态方面发生早衰的可能性，极有可能是由于cuzn -超氧化物歧化酶的基因剂量效应。
Transgenic mice carrying the human CuZn-superoxide dismutase gene were used to investigate whether CuZn-superoxide dismutase gene dosage is involved in the signs of neuromuscular junction deterioration associated with Down's syndrome. Three parameters of neuromuscular junction morphology were studied in hindlimb muscles of CuZn-superoxide dismutase-transgenic mice and their non-transgenic littermates: nerve terminal length, number of nerve terminal branching points and incidence of sprouting that results in synapse formation. These parameters increased with advanced age and the increase occurred earlier in CuZn-superoxide dismutase-transgenic mice. Therefore, the data is in line with the possibility that CuZn-superoxide dismutase-transgenic mice are undergoing premature ageing with respect to neuromuscular junction morphology, most probably owing to a gene dosage effect of CuZn-superoxide dismutase.