Progress on low susceptibility mechanisms of transmissible spongiform encephalopathies

Dongwuxue Yanjiu. 2014 Sep;35(5):436-45. doi: 10.13918/j.issn.2095-8137.2014.5.436.

Abstract

Transmissible spongiform encephalopathies (TSEs), also known as prion diseases, are a group of fatal neurodegenerative diseases detected in a wide range of mammalian species. The "protein-only" hypothesis of TSE suggests that prions are transmissible particles devoid of nucleic acid and the primary pathogenic event is thought to be the conversion of cellular prion protein (PrP(C)) into the disease-associated isoform (PrP(Sc)). According to susceptibility to TSEs, animals can be classified into susceptible species and low susceptibility species. In this review we focus on several species with low susceptibility to TSEs: dogs, rabbits, horses and buffaloes. We summarize recent studies into the characteristics of low susceptibility regarding protein structure, and biochemical and genetic properties.

Keywords: Buffalo; Dog; Horse; Low susceptibility; PRNP; Rabbit; SPRN; Transmissible spongiform encephalopathy.

Publication types

  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Amino Acid Sequence
  • Animals
  • Molecular Sequence Data
  • Prion Diseases / genetics
  • Prion Diseases / veterinary*
  • Prions / chemistry
  • Prions / genetics
  • Prions / metabolism*
  • Species Specificity

Substances

  • Prions

Grants and funding

This item was supported by the National Natural Science Foundation of China (31060302 and 31260032), the Transgene Special Project of the Ministry of Agriculture of China (2011ZX08009-003-006) and the Natural Science Foundation of Yunnan Province (2010CD010)