Structural effects of the highly protective V127 polymorphism on human prion protein.
Communications Biology
Hosszu, Laszlo L P LLP; Conners, Rebecca R; Sangar, Daljit D; Batchelor, Mark M; Sawyer, Elizabeth B EB; Fisher, Stuart S; Cliff, Matthew J MJ; Hounslow, Andrea M AM; McAuley, Katherine K; Leo Brady, R R; Jackson, Graham S GS; Bieschke, Jan J; Waltho, Jonathan P JP; Collinge, John J
mTOR Signaling and SREBP Activity Increase FADS2 Expression and Can Activate Sapienate Biosynthesis.
Cell Reports
Triki, Mouna M; Rinaldi, Gianmarco G; Planque, Melanie M; Broekaert, Dorien D; Winkelkotte, Alina M AM; Maier, Carina R CR; Janaki Raman, Sudha S; Vandekeere, Anke A; Van Elsen, Joke J; Orth, Martin F MF; Grünewald, Thomas G P TGP; Schulze, Almut A; Fendt, Sarah-Maria SM
Infectious Disease Transmission in Solid Organ Transplantation: Donor Evaluation, Recipient Risk, and Outcomes of Transmission.
Transplantation Direct
White, Sarah L SL; Rawlinson, William W; Boan, Peter P; Sheppeard, Vicky V; Wong, Germaine G; Waller, Karen K; Opdam, Helen H; Kaldor, John J; Fink, Michael M; Verran, Deborah D; Webster, Angela A; Wyburn, Kate K; Grayson, Lindsay L; Glanville, Allan A; Cross, Nick N; Irish, Ashley A; Coates, Toby T; Griffin, Anthony A; Snell, Greg G; Alexander, Stephen I SI; Campbell, Scott S; Chadban, Steven S; Macdonald, Peter P; Manley, Paul P; Mehakovic, Eva E; Ramachandran, Vidya V; Mitchell, Alicia A; Ison, Michael M
Impaired transmissibility of atypical prions from genetic CJDG114V.
Neurology. Genetics
Cali, Ignazio I; Mikhail, Fadi F; Qin, Kefeng K; Gregory, Crystal C; Solanki, Ani A; Martinez, Manuel Camacho MC; Zhao, Lili L; Appleby, Brian B; Gambetti, Pierluigi P; Norstrom, Eric E; Mastrianni, James A JA
Familial human prion diseases associated with prion protein mutations Y226X and G131V are transmissible to transgenic mice expressing human prion protein.
Acta Neuropathologica Communications
Race, Brent B; Williams, Katie K; Hughson, Andrew G AG; Jansen, Casper C; Parchi, Piero P; Rozemuller, Annemieke J M AJM; Chesebro, Bruce B
Genetic prion disease: Experience of a rapidly progressive dementia center in the United States and a review of the literature.
American Journal Of Medical Genetics. Part B, Neuropsychiatric Genetics : The Official Publication Of The International Society Of Psychiatric Genetics
Takada, Leonel T LT; Kim, Mee-Ohk MO; Cleveland, Ross W RW; Wong, Katherine K; Forner, Sven A SA; Gala, Ignacio Illán II; Fong, Jamie C JC; Geschwind, Michael D MD
Activation of the unfolded protein response and granulovacuolar degeneration are not common features of human prion pathology.
Acta Neuropathologica Communications
Wiersma, Vera I VI; van Hecke, Wim W; Scheper, Wiep W; van Osch, Martijn A J MA; Hermsen, Will J M WJ; Rozemuller, Annemieke J M AJ; Hoozemans, Jeroen J M JJ
Human prion diseases in the Netherlands (1998-2009): clinical, genetic and molecular aspects.
Plos One
Jansen, Casper C; Parchi, Piero P; Capellari, Sabina S; Ibrahim-Verbaas, Carla A CA; Schuur, Maaike M; Strammiello, Rosaria R; Corrado, Patrizia P; Bishop, Matthew T MT; van Gool, Willem A WA; Verbeek, Marcel M MM; Baas, Frank F; van Saane, Wesley W; Spliet, Wim G M WG; Jansen, Gerard H GH; van Duijn, Cornelia M CM; Rozemuller, Annemieke J M AJ
Crystallographic studies of prion protein (PrP) segments suggest how structural changes encoded by polymorphism at residue 129 modulate susceptibility to human prion disease.
The Journal Of Biological Chemistry
Apostol, Marcin I MI; Sawaya, Michael R MR; Cascio, Duilio D; Eisenberg, David D
Prion proteins with different conformations accumulate in Gerstmann-Sträussler-Scheinker disease caused by A117V and F198S mutations.
The American Journal Of Pathology
Piccardo, P P; Liepnieks, J J JJ; William, A A; Dlouhy, S R SR; Farlow, M R MR; Young, K K; Nochlin, D D; Bird, T D TD; Nixon, R R RR; Ball, M J MJ; DeCarli, C C; Bugiani, O O; Tagliavini, F F; Benson, M D MD; Ghetti, B B