Prion protein expression in muscle cells and toxicity of a prion proteinfragment

Brown DR.; Schmidt B.; Groschup MH.; Kretzschmar HA.

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Prion protein expression in muscle cells and toxicity of a prion proteinfragment

机译：muscle肌蛋白在肌肉细胞中的表达和toxicity蛋白片段的毒性

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The prion protein (PrP) is a cell surface glycoprotein normally associated with neurones. Expression of the prion protein in cultured mouse myoblasts and myotubes suggests that the prion protein may play a physiological role in skeletal muscle. When myotubes differentiate from myoblasts prion protein expression is upregulated. Accompanying this increase is an upregulation of Cu/Zn superoxide dismutase (SOD-1) in myotubes. Muscle cells derived from mice deficient in cellular PrP (PrPC) show little increase in SOD-1 after differentiation from myoblasts to myotubes. Myoblasts and myotubes are resistant to the toxicity of a neurotoxic prion protein peptide (PrP106-126). However, in the presence of murine microglia, PrP106-126 causes a reduction in cell number. This effect is greater on myotubes than myoblasts. Even in the presence of microglia PrP106-126 is not toxic to muscle cells derived from PrP-deficient mice. Our results suggest that PrPC expression is associated with regulation of cellular resistance to oxidative stress in skeletal muscle.

机译：ion病毒蛋白（PrP）是通常与神经元相关的细胞表面糖蛋白。 mouse病毒蛋白在培养的小鼠成肌细胞和肌管中的表达表明the病毒蛋白可能在骨骼肌中发挥生理作用。当肌管与成肌细胞分化时，病毒蛋白表达上调。伴随这种增加的是肌管中Cu / Zn超氧化物歧化酶（SOD-1）的上调。从成肌细胞分化为肌管后，缺乏细胞PrP（PrPC）的小鼠衍生的肌肉细胞显示SOD-1几乎没有增加。成肌细胞和肌管对神经毒性病毒蛋白肽（PrP106-126）的毒性具有抗性。然而，在鼠小胶质细胞的存在下，PrP106-126导致细胞数量减少。这种作用在肌管上比成肌细胞更大。即使在存在小胶质细胞的情况下，PrP106-126对衍生自PrP缺陷小鼠的肌肉细胞也没有毒性。我们的结果表明，PrPC表达与细胞对骨骼肌氧化应激的抗性调控有关。

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《European Journal of Cell Biology: Journal of Deutsche Gesellschaft fur Elektronenmikroskopie: Journal of Deutsche Gesellschaft fur Zellbiologie》 |1998年第1期|共9页
作者
Brown DR.; Schmidt B.; Groschup MH.; Kretzschmar HA.;
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正文语种 eng
中图分类细胞生物学;
关键词
Prion protein; Muscle; Neurotoxicity; Oxidative stress; Receptor-inducing activity; Inclusion-body myositis; Scrapie prion; Messenger-rna; Mice lacking; Gene; Prp; Diseases; Brain; Neurotoxicity;

机译：on病毒蛋白;肌肉;神经毒性;氧化应激;受体诱导活性;包涵体肌炎;S病毒;Messenger-rna;小鼠;基因;Prp;疾病;脑;神经毒性;

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专利

1. Prion protein expression in muscle cells and toxicity of a prion proteinfragment [J] . Brown DR., Schmidt B., Groschup MH., European Journal of Cell Biology: Journal of Deutsche Gesellschaft fur Elektronenmikroskopie: Journal of Deutsche Gesellschaft fur Zellbiologie . 1998,第1期

机译：muscle肌蛋白在肌肉细胞中的表达和toxicity蛋白片段的毒性
2. Association of prion protein genotype and scrapie prion protein type with cellular prion protein charge isoform profiles in cerebrospinal fluid of humans with sporadic or familial prion diseases [J] . SchmitzM., LüllmannK., ZafarS., Neurobiology of Aging: Experimental and Clinical Research . 2014,第5期

机译：散发性或家族性病毒病患者脑脊液中病毒蛋白基因型和瘙痒病pr病毒蛋白类型与细胞病毒蛋白电荷同工型的关系
3. Overexpression of quality control proteins reduces prion conversion in prion-infected cells [J] . Simrika Thapa, Basant Abdulrahman, Dalia H. Abdelaziz, The Journal of biological chemistry . 2018,第41期

机译：质量控制蛋白的过表达减少了病毒感染细胞中的ion病毒转化
4. New insights into Doppel neurotoxicity using cerebellar organotypic cultures from prion protein-deficient mice. [C] . Dole S, Heitz S, Bombarde G, International PRION Congress . 2010

机译：朊病毒蛋白质缺陷小鼠小脑有机型培养物对Doppel神经毒性的新见解。
5. Neurobehavioral impairments of prion protein deficient mice in olfactory tasks: Towards a functional understanding of the cellular prion protein PrP(C). [D] . Le Pichon, Claire. 2007

机译：嗅觉任务中的ion病毒蛋白缺陷小鼠的神经行为损害：对细胞病毒蛋白PrP（C）的功能性理解。
6. Cytosolic Prion Protein Toxicity Is Independent of Cellular Prion Protein Expression and Prion Propagation [O] . Eric M. Norstrom, Mark F. Ciaccio, Benjamin Rassbach, 2007

机译：胞质Pri蛋白的毒性独立于细胞on蛋白的表达和Pri子的繁殖
7. Cytosolic Prion Protein Toxicity Is Independent of Cellular Prion Protein Expression and Prion Propagation▿ [O] . Norstrom, Eric M., Ciaccio, Mark F., Rassbach, Benjamin, 2006

机译：胞质Pri蛋白的毒性独立于细胞on蛋白的表达和on蛋白的繁殖▿

Prion protein expression in muscle cells and toxicity of a prion proteinfragment

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