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mdx中抗肌萎缩蛋白阳性纤维的发育研究及动力相关蛋白定位

Developmental studies of dystrophin-positive fibers in mdx, and DRP localization.

作者信息

Zhao J, Yoshioka K, Miike T, Miyatake M

机构信息

Department of Child Development, Kumamoto University Medical School, Japan.

出版信息

J Neurol Sci. 1993 Jan;114(1):104-8. doi: 10.1016/0022-510x(93)90056-5.

DOI:10.1016/0022-510x(93)90056-5
PMID:8433092
Abstract

Dystrophin positive fibers (DPFs) were observed in about 1% of the total muscle fibers in 1-year-old mice. Some of these fibers were found to have positive staining with all six antibodies, while others showed a negative reaction with specific antibodies. These results suggest that the most likely mechanism giving rise to these DPFs is a second site mutation which prepares in-frame deletion. A study of the frequency of DPF during development showed single and scattered DPFs in younger mice, which gradually increased in number and began to form small groups with age. DRP was observed constantly on the neuromuscular junctions in both control and mdx muscle, and surface membrane of immature muscle fibers such as regenerating fibers in mdx and newborn muscle during 2 weeks of age in control and mdx.

摘要

在1岁小鼠的全部肌纤维中,约1%观察到抗肌萎缩蛋白阳性纤维(DPF)。这些纤维中的一些被发现对所有六种抗体均呈阳性染色,而其他纤维对特定抗体呈阴性反应。这些结果表明,产生这些DPF最可能的机制是导致框内缺失的第二位点突变。一项关于发育过程中DPF频率的研究表明,年幼小鼠中有单个且分散的DPF,其数量随年龄逐渐增加,并开始形成小群体。在对照和mdx肌肉的神经肌肉接头处以及未成熟肌纤维的表面膜上持续观察到DRP,如mdx中的再生纤维以及对照和mdx中2周龄新生肌肉中的未成熟肌纤维表面膜。

相似文献

1
Developmental studies of dystrophin-positive fibers in mdx, and DRP localization.mdx中抗肌萎缩蛋白阳性纤维的发育研究及动力相关蛋白定位
J Neurol Sci. 1993 Jan;114(1):104-8. doi: 10.1016/0022-510x(93)90056-5.
2
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引用本文的文献

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Electroporation Enhanced Effect of Dystrophin Splice Switching PNA Oligomers in Normal and Dystrophic Muscle.电穿孔增强抗肌萎缩蛋白剪接转换肽核酸寡聚体在正常和营养不良肌肉中的作用。
Mol Ther Nucleic Acids. 2015 Dec 1;4(12):e267. doi: 10.1038/mtna.2015.41.
2
Extensive but coordinated reorganization of the membrane skeleton in myofibers of dystrophic (mdx) mice.营养不良(mdx)小鼠肌纤维中膜骨架的广泛但协调的重组。
J Cell Biol. 1999 Mar 22;144(6):1259-70. doi: 10.1083/jcb.144.6.1259.
3
Successful histocompatible myoblast transplantation in dystrophin-deficient mdx mouse despite the production of antibodies against dystrophin.
尽管产生了抗肌营养不良蛋白的抗体,但在肌营养不良蛋白缺陷的mdx小鼠中成功进行了组织相容性成肌细胞移植。
J Cell Biol. 1995 Nov;131(4):975-88. doi: 10.1083/jcb.131.4.975.