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髓鞘缺陷型颤抖突变小鼠中枢神经系统中2',3'-环核苷酸3'-磷酸水解酶的亚细胞分布及发育变化

Subcellular distribution and developmental change of 2',3'-cyclic nucleotide 3'-phosphohydrolase in the central nervous system of the myelin-deficient shiverer mutant mice.

作者信息

Mikoshiba K, Nagaike K, Tsukada Y

出版信息

J Neurochem. 1980 Aug;35(2):465-70. doi: 10.1111/j.1471-4159.1980.tb06288.x.

Abstract

Shiverer is an autosomal recessive mutant characterized by dysmyelination in the central nervous system. The myelin in the shiverer is devoid of the major dense line. The pattern of increase of 2',3'-cyclic nucleotide 3'-phosphohydrolase (CNPase) in the various parts of central nervous system during postnatal development was identical with that of controls. Subcellular fractionation of the brain from shiverer showed that the recovery of CNPase activity of the P2A was only 14% of the control, but those of P2B and P3 were several times higher than the controls. The specificity activity of CNPase of the purified myelin and P2A from shiverer did not differ significantly from that of the controls. The combined P2 and P3 (P2 + P3) fraction was subjected to linear sucrose density gradient (0.32--1.3 M) centrifugation. By measuring CNPase activities of the fractions obtained after centrifugation, shiverer showed two peaks of CNPase activities in the sucrose gradient, although that of the control showed a single peak. The main peak obtained from shiverer was found in the region of higher sucrose concentration than that of the control, while a small peak was found in the region of lower sucrose concentration.

摘要

颤抖鼠是一种常染色体隐性突变体,其特征是中枢神经系统髓鞘形成异常。颤抖鼠的髓鞘缺乏主要致密线。在出生后发育过程中,中枢神经系统各部位2',3'-环核苷酸3'-磷酸水解酶(CNPase)的增加模式与对照组相同。对颤抖鼠大脑进行亚细胞分级分离显示,P2A部分的CNPase活性回收率仅为对照组的14%,但P2B和P3部分的活性回收率比对照组高几倍。从颤抖鼠纯化的髓鞘和P2A中CNPase的比活性与对照组相比无显著差异。将合并的P2和P3(P2 + P3)部分进行线性蔗糖密度梯度(0.32 - 1.3 M)离心。通过测量离心后各部分的CNPase活性,颤抖鼠在蔗糖梯度中显示出两个CNPase活性峰,而对照组显示出一个单峰。从颤抖鼠获得的主峰位于比对照组更高蔗糖浓度区域,而在较低蔗糖浓度区域发现一个小峰。

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