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泼尼松龙可增强mdx小鼠骨骼肌细胞培养物中的肌生成和抗肌萎缩蛋白相关蛋白。

Prednisolone enhances myogenesis and dystrophin-related protein in skeletal muscle cell cultures from mdx mouse.

作者信息

Passaquin A C, Metzinger L, Léger J J, Warter J M, Poindron P

机构信息

Département d'Immunologie, Immunopharmacologie et Pathologie, Université Louis Pasteur, Illkirch, France.

出版信息

J Neurosci Res. 1993 Jul 1;35(4):363-72. doi: 10.1002/jnr.490350403.

DOI:10.1002/jnr.490350403
PMID:8360945
Abstract

The differentiation of skeletal muscle cells from mdx mice which lack dystrophin expression was examined after glucocorticoid treatment, namely alpha-methylprednisolone (PDN). Primary skeletal muscle cell cultures were established from newborn mdx, congenic C57BL/10, and allogenic BALB/C mice. We show that PDN promotes the myogenesis of both mdx- and control mice-derived cultures as determined by 1) the number of myotubes, 2) acetylcholine receptors, and 3) dystrophin and dystrophin-related protein levels. These results support the hypothesis that PDN could enhance the myogenesis of satellite cells and increase dystrophin-related protein expression in DMD treated patients.

摘要

在给予糖皮质激素治疗(即α-甲基泼尼松龙,PDN)后,研究了缺乏抗肌萎缩蛋白表达的mdx小鼠骨骼肌细胞的分化情况。从新生mdx小鼠、同基因C57BL/10小鼠和同种异体BALB/C小鼠建立了原代骨骼肌细胞培养物。我们发现,通过以下三个指标确定,PDN可促进mdx小鼠和对照小鼠来源培养物的肌生成:1)肌管数量;2)乙酰胆碱受体;3)抗肌萎缩蛋白和抗肌萎缩蛋白相关蛋白水平。这些结果支持这样的假说,即PDN可增强卫星细胞的肌生成,并增加杜氏肌营养不良症(DMD)患者中抗肌萎缩蛋白相关蛋白的表达。

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1
Prednisolone enhances myogenesis and dystrophin-related protein in skeletal muscle cell cultures from mdx mouse.泼尼松龙可增强mdx小鼠骨骼肌细胞培养物中的肌生成和抗肌萎缩蛋白相关蛋白。
J Neurosci Res. 1993 Jul 1;35(4):363-72. doi: 10.1002/jnr.490350403.
2
Expression of full-length utrophin prevents muscular dystrophy in mdx mice.全长抗肌萎缩蛋白的表达可预防mdx小鼠的肌肉萎缩症。
Nat Med. 1998 Dec;4(12):1441-4. doi: 10.1038/4033.
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alpha-Methylprednisolone promotes skeletal myogenesis in dystrophin-deficient and control mouse cultures.α-甲基泼尼松龙可促进肌营养不良蛋白缺陷型和对照小鼠培养物中的骨骼肌生成。
Neurosci Lett. 1993 Jun 11;155(2):171-4. doi: 10.1016/0304-3940(93)90700-u.
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Skeletal muscle-specific expression of a utrophin transgene rescues utrophin-dystrophin deficient mice.肌动蛋白转基因在骨骼肌中的特异性表达挽救了肌动蛋白-抗肌萎缩蛋白缺陷小鼠。
Nat Genet. 1998 May;19(1):79-82. doi: 10.1038/ng0598-79.
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Dystroglycan mRNA expression during normal and mdx mouse embryogenesis: a comparison with utrophin and the apo-dystrophins.正常及mdx小鼠胚胎发育过程中肌营养不良聚糖mRNA的表达:与抗肌萎缩蛋白及脱辅基肌营养不良蛋白的比较
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Dystroglycan expression in the wild type and mdx mouse neural retina: synaptic colocalization with dystrophin, dystrophin-related protein but not laminin.野生型和mdx小鼠神经视网膜中肌营养不良聚糖的表达:与肌营养不良蛋白、肌营养不良相关蛋白而非层粘连蛋白的突触共定位。
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Expression of a NOS transgene in dystrophin-deficient muscle reduces muscle membrane damage without increasing the expression of membrane-associated cytoskeletal proteins.在缺乏肌营养不良蛋白的肌肉中,一氧化氮合酶转基因的表达可减少肌膜损伤,而不会增加膜相关细胞骨架蛋白的表达。
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Amelioration of the dystrophic phenotype of mdx mice using a truncated utrophin transgene.使用截短的抗肌萎缩蛋白转基因改善mdx小鼠的营养不良表型。
Nature. 1996 Nov 28;384(6607):349-53. doi: 10.1038/384349a0.
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Smooth muscle-specific dystrophin expression improves aberrant vasoregulation in mdx mice.平滑肌特异性肌营养不良蛋白表达改善了mdx小鼠异常的血管调节。
Hum Mol Genet. 2006 Jul 15;15(14):2266-75. doi: 10.1093/hmg/ddl151. Epub 2006 Jun 15.
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Dystrophin-related protein is found in the central nervous system of mice at various developmental stages, especially at the postsynaptic membrane.肌营养不良蛋白相关蛋白在小鼠不同发育阶段的中枢神经系统中均有发现,尤其是在突触后膜。
J Neurosci Res. 1994 Apr 15;37(6):728-34. doi: 10.1002/jnr.490370607.

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