中国神经再生研究(英文版) ›› 2018, Vol. 13 ›› Issue (12): 2108-2110.doi: 10.4103/1673-5374.241460

• 观点:退行性病与再生 • 上一篇    下一篇

肽适体介导的朊病毒蛋白α切割调节作为朊病毒和其他神经退行性疾病的治疗策略

  

  • 收稿日期:2018-07-18 出版日期:2018-12-15 发布日期:2018-12-15

Peptide aptamer-mediated modulation of prion protein α-cleavage as treatment strategy for prion and other neurodegenerative diseases

Antonia N. Klein1, 2, 3, Erica Corda, Sabine Gilch1, 2, 3   

  1. 1 Department of Ecosystem and Public Health, Faculty of Veterinary Medicine, University of Calgary, Calgary, Alberta, Canada;
    2 Calgary Prion Research Unit, University of Calgary, Calgary, Alberta, Canada;
    3 Hotchkiss Brain Insti¬tute, University of Calgary, Calgary, Alberta, Canada
  • Received:2018-07-18 Online:2018-12-15 Published:2018-12-15
  • Contact: Sabine Gilch, PhD, sgilch@ucalgary.ca.
  • Supported by:

    This work was funded by grants from the Alberta Prion Research Institute, the Alzheimer Society of Alberta and Northwest Territories and the Natural Sciences and Engineering Research Council (NSERC) of Canada. SG is supported by the Canada Research Chair program, ANK received a postdoctoral fellowship from the German Research Foundation (DFG).

摘要:

orcid: 0000-0001-5923-3464 (Sabine Gilch)

Abstract:

Despite intensive research, most neurodegenerative diseases cannot be cured and for some of them no treatment is available to increase survival or quality of life. Among the latter are prion diseases, fatal and transmissible neurodegenerative diseases of humans and other animals. Examples are Creutzfeldt-Jakob disease (CJD) in man, bovine spongiform encephalopathy (BSE, also known as mad cow disease) in cattle, scrapie in sheep and goats, and chronic wasting disease (CWD) in cervids. Most human prion diseases manifest sporadically, but also genetic and infectious origins are known. Prions, the causal agent of prion diseases, are composed solely of protein, namely a misfolded isoform of the cellular prion protein PrPC, termed PrPSc. They are transmissible within and between species. Human-to-human transmission can occur through medical procedures (e.g.,neurosurgery) and results in iatrogenic CJD (iCJD). The largest number of iCJD cases worldwide has been reported upon treating growth hormone deficits with prion-contaminated cadaveric pituitary-derived human growth hormones. Transmission of BSE from cows to humans is to date the only example of zoonotic prion transmission resulting in variant CJD. Whether CWD is transmissible to humans is unknown but represents a current threat due to expansion of geographic distribution within and beyond North America and in light of a novel study indicating oral transmission to non-human primates. In this context, finding a treatment against prion disease is of primary importance.