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

Progress on low susceptibility mechanisms of transmissible spongiform encephalopathies

Li-Li Qing  1 , Hui Zhao  2 , Lin-Lin Liu  1
Affiliations
Review

Progress on low susceptibility mechanisms of transmissible spongiform encephalopathies

Li-Li Qing et al. Dongwuxue Yanjiu. 2014 Sep.

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.

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Conflict of interest statement

The authors have declared that no competing interests exist.

Figures

Figure 1
Figure 1
Amino acid sequences of PrP in 16 mammals (data from GenBank) Human (NM_000311.3), chimpanzee (NM_001009093.3), Rhesus (NM_001047152.1), deer (AY330343.1), elk (EU082291.1), mouse (NM_011170.3), rat (NM_012631.2), pig (NM_001008687.1), sheep (NM_001009481.1), goat(JF729302.1), rabbit (NM_001082021.1), dog (NM_001013423.1), cat (EU341499.1), horse(NM_001143798.1), cattle (NM_181015.2), buffalo ( KC.1). 137634.
Figure 2
Figure 2
Propensities of conversions of PrPC into the β intermediate state in different species at pH 7.0 (A), 5.0 (B), 4.5 (C) and 4.0 (D) and different concentrations of urea (modified from Khan et al, 2010)
See this image and copyright information in PMC

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