期刊文献+

Advances in research on Shadoo, shadow of prion protein 被引量:3

Advances in research on Shadoo, shadow of prion protein
原文传递
导出
摘要 Prion plays a central role in the pathogenesis of transmissible spongiform encephalopathies,also known as prion diseases.However,the biology of the protein and the pathophysiology of these diseases remain largely unknown.It has been speculated that additional factor or factors may be involved in the pathogenesis of prion diseases.Recently,a PrP-like protein,recognized as shadow of prion protein(Shadoo,Sho),is thought to be an interesting candidate factor because both the prion protein and Sho have been shown to have overlapping expression patterns and shared functions.Therefore,extensive study of Sho may advance our understanding of the enigmatical biology of prion and prion diseases.In this review,recent studies on Sho associated with prion diseases and functions are summarized.These studies have demonstrated the functional importance of Sho,and they further need to investigate its biological roles in prion diseases. Prion plays a central role in the pathogenesis of transmissible spongiform encephalopathies, also known as prion diseases. However, the biology of the protein and the pathophysiology of these diseases remain largely unknown. It has been speculated that additional factor or factors may be involved in the pathogenesis of prion diseases. Recently, a PrP-like protein, recognized as shadow of prion protein (Shadoo, Sho), is thought to be an interesting candidate factor because both the prion protein and Sho have been shown to have overlapping expression patterns and shared functions. Therefore, extensive study of Sho may advance our understanding of the enigmatical biology of prion and prion diseases. In this review, recent studies on Sho asso- ciated with prion diseases and functions are summarized. These studies have demonstrated the functional importance of Sho, and they further need to investigate its biological roles in prion diseases.
出处 《Chinese Science Bulletin》 SCIE EI CAS 2014年第9期821-827,共7页
基金 supported by the National Natural Science Foundation of China(31060302,31260032) the Natural Science Foundation of Yunnan Province(2010CD010)
关键词 阴影 朊蛋白 传染性海绵状脑病 生物学作用 朊病毒病 朊病毒蛋白 朊病毒疾病 发病机制 Shadoo Prion Prion disease - FunctionPolymorphism Expression
  • 相关文献

参考文献63

  • 1Prusiner SB (1998) Prions. Proc Natl Acad Sci USA 95: 13363-13383.
  • 2Brandner S, Raeber A, Sailer A et al (1996) Normal host prion protein (PrPc) is required for scrapie spread within the central nervous system. Proc Natl Acad Sci USA 93:13148-13151.
  • 3Richt JA, Kasinathan P, Hamir AN et a| (2007) Production of cattle lacking prion protein. Nat Biotechnol 25:132-138.
  • 4Bueler H, Fischer M, Lang Yet al (1992) Normal development and behaviour of mice lacking the neuronal cell-surface PrP protein. Nature 356:577-582.
  • 5Manson JC, Clarke AR, Hooper ML et al (1994) 129/Ola mice carrying a null mutation in PrP that abolishes mRNA production are developmentally normal. Mol Neurobiol 8:121-127.
  • 6Yu G, Chen J, Xu Y et al (2009) Generation of goats lacking prion protein. Mol Reprod 76:3-12.
  • 7Benestad SL, Austbo L, Tranulis MA et al (2012) Healthy goats naturally devoid of prion protein. Vet Res 43:87.
  • 8Premzl M, Sangiorgio L, Strumbo Bet al (2003) Shadoo, a new protein highly conserved from fish to mammals and with simi- larity to prion protein. Gene 314:89-102.
  • 9Silverman GL, Qin K, Moore RC et al (2000) Doppel is an N- glycosylated, glycosylphosphatidylinositol-anchored protein. Expression in testis and ectopic production in the brains of Prnp(1/1) mice predisposed to Purkinje cell loss. J Biol Chem 275: 26834-26841.
  • 10Behrens A, Genoud N, Naumann H et al (2002) Absence of the prion protein bomologue Doppel causes real sterility. EMBO J 21:3652-3658.

同被引文献25

引证文献3

二级引证文献1

相关作者

内容加载中请稍等...

相关机构

内容加载中请稍等...

相关主题

内容加载中请稍等...

浏览历史

内容加载中请稍等...
;
使用帮助 返回顶部