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- W3071473169 abstract "Prion diseases are a group of neurodegenerative diseases endemic in humans and several ruminants caused by the misfolding of native prion protein (PrP) into pathological conformations. Experimental work and the mad-cow epidemic of the 1980’s exposed a wide spectrum of animal susceptibility to prion diseases, including a few highly resistant animals: horses, rabbits, pigs, and dogs / canids. The variable susceptibility to disease offers a unique opportunity to uncover the mechanisms governing PrP misfolding, neurotoxicity, and transmission. Previous work indicates that PrP-intrinsic differences (sequence) are the main contributors to disease susceptibility. Several residues have been cited as critical for encoding PrP conformational stability in prion-resistant animals, including D/E159 in dog, S167 in horse, and S174 in rabbit and pig PrP (all according to human numbering). These amino acids alter PrP properties in a variety of assays, but we still do not clearly understand the structural correlates of PrP toxicity. Additional insight can be extracted from comparative structural studies, followed by molecular dynamics simulations of selected mutations, and testing in manipulable animal models. Our working hypothesis is that protective amino acids generate more compact and stable structures in a C-terminal subdomain of the PrP globular domain. We will explore this idea in this review and identify subdomains within the globular domain that may hold the key to unravel how conformational stability and disease susceptibility are encoded in PrP." @default.
- W3071473169 created "2020-08-24" @default.
- W3071473169 creator A5023798163 @default.
- W3071473169 creator A5053029811 @default.
- W3071473169 creator A5071239555 @default.
- W3071473169 date "2020-08-18" @default.
- W3071473169 modified "2023-10-16" @default.
- W3071473169 title "Insight From Animals Resistant to Prion Diseases: Deciphering the Genotype – Morphotype – Phenotype Code for the Prion Protein" @default.
- W3071473169 cites W1087224093 @default.
- W3071473169 cites W137320127 @default.
- W3071473169 cites W1482305165 @default.
- W3071473169 cites W1563777667 @default.
- W3071473169 cites W1591864042 @default.
- W3071473169 cites W1595632545 @default.
- W3071473169 cites W1900496951 @default.
- W3071473169 cites W1938969372 @default.
- W3071473169 cites W1963941072 @default.
- W3071473169 cites W1972002560 @default.
- W3071473169 cites W1976321523 @default.
- W3071473169 cites W1978157456 @default.
- W3071473169 cites W1980075338 @default.
- W3071473169 cites W1980237498 @default.
- W3071473169 cites W1986735189 @default.
- W3071473169 cites W1987247710 @default.
- W3071473169 cites W1990782428 @default.
- W3071473169 cites W1991934962 @default.
- W3071473169 cites W1992380356 @default.
- W3071473169 cites W1995198702 @default.
- W3071473169 cites W1996947727 @default.
- W3071473169 cites W1997944660 @default.
- W3071473169 cites W1999301383 @default.
- W3071473169 cites W1999802354 @default.
- W3071473169 cites W2001763373 @default.
- W3071473169 cites W2001825719 @default.
- W3071473169 cites W2004666307 @default.
- W3071473169 cites W2005969043 @default.
- W3071473169 cites W2006431604 @default.
- W3071473169 cites W2006915853 @default.
- W3071473169 cites W2008091234 @default.
- W3071473169 cites W2012541956 @default.
- W3071473169 cites W2013215890 @default.
- W3071473169 cites W2015774470 @default.
- W3071473169 cites W2016656104 @default.
- W3071473169 cites W2016752687 @default.
- W3071473169 cites W2018407130 @default.
- W3071473169 cites W2018675827 @default.
- W3071473169 cites W2019007228 @default.
- W3071473169 cites W2021173114 @default.
- W3071473169 cites W2023529808 @default.
- W3071473169 cites W2026093694 @default.
- W3071473169 cites W2036675767 @default.
- W3071473169 cites W2039168977 @default.
- W3071473169 cites W2044567938 @default.
- W3071473169 cites W2049627175 @default.
- W3071473169 cites W2051632057 @default.
- W3071473169 cites W2052091508 @default.
- W3071473169 cites W2053699160 @default.
- W3071473169 cites W2057587849 @default.
- W3071473169 cites W2059804089 @default.
- W3071473169 cites W2064217705 @default.
- W3071473169 cites W2075606263 @default.
- W3071473169 cites W2076146345 @default.
- W3071473169 cites W2077572475 @default.
- W3071473169 cites W2080488591 @default.
- W3071473169 cites W2083642579 @default.
- W3071473169 cites W2083715113 @default.
- W3071473169 cites W2090577984 @default.
- W3071473169 cites W2096963432 @default.
- W3071473169 cites W2100264046 @default.
- W3071473169 cites W2103352856 @default.
- W3071473169 cites W2108896069 @default.
- W3071473169 cites W2109401365 @default.
- W3071473169 cites W2115925208 @default.
- W3071473169 cites W2117388141 @default.
- W3071473169 cites W2120316898 @default.
- W3071473169 cites W2132346258 @default.
- W3071473169 cites W2137067600 @default.
- W3071473169 cites W2138468217 @default.
- W3071473169 cites W2138768135 @default.
- W3071473169 cites W2141169243 @default.
- W3071473169 cites W2143795630 @default.
- W3071473169 cites W2150151638 @default.
- W3071473169 cites W2153526379 @default.
- W3071473169 cites W2153865022 @default.
- W3071473169 cites W2162802187 @default.
- W3071473169 cites W2166824294 @default.
- W3071473169 cites W2234012305 @default.
- W3071473169 cites W2324660492 @default.
- W3071473169 cites W2329424922 @default.
- W3071473169 cites W2434347899 @default.
- W3071473169 cites W2484566956 @default.
- W3071473169 cites W2564670120 @default.
- W3071473169 cites W2576097146 @default.
- W3071473169 cites W2579037333 @default.
- W3071473169 cites W2605844712 @default.
- W3071473169 cites W2745294757 @default.
- W3071473169 cites W2745333017 @default.
- W3071473169 cites W2762937225 @default.
- W3071473169 cites W2769529271 @default.
- W3071473169 cites W2781208504 @default.