1.College of Animal Science and Veterinary Medicine, Jilin University, Changchun 130062, China; 2.The Military Veterinary Institute, Academy of Military Medical Science of PLA, Changchun 130122, China; 3. Key Laboratory of Jilin Province for Zoonosis Prevention and Control, Changchun 130122, China; 4. The Forth Hospital of Jilin University, Changchun 130011, China
Abstract:The prion hypothesis posits that a misfolded form of prion protein (PrP) is responsible for the infectivity of prion disease. After intracerebral injection of the 22L strain of scrapie, wild-type mice developed neurological signs in 130 day and reached the terminal stage of disease in 150 day. The typical microscopic features of TSEs were vacuolation of the neuropil in the gray matter, prominent neuronal loss, exuberant reactive astrogliosis, and a variable degree of cerebral accumulation of prion protein aggregates.
[1] Prusiner SB. Prions causing degenerative neurological diseases[J] . Annu Rev Med, 1987, 38: 381-398. DOI: 10.1146/annurev.me.38.020187.002121 [2] Saa P, Castilla J, Soto C, et al. Ultra-efficient replication of infectious prions by automated protein misfolding cyclic amplification[J] . J Biol Chem, 2006, 281(46): 35245-35252. DOI: 10.1074/jbc.M603964200 [3] Tattum MH, Jones S, Pal S, et al. Discrimination between prion-infected and normal blood samples by protein misfolding cyclic amplification[J] . Transfusion,2010, 50(5): 996-1002. DOI:10.1111/j.1537-2995.2010.02595.x [4] Linden R, Martins VR, Prado M, et al. Physiology of the prion protein[J] . Physiol Rev, 2008, 88(2): 673-728.DOI:10.1152/physrev.00007.2007 [5] Watts JC, Drisaldi B, Ng V, et al. The CNS glycoprotein Shadoo has PrPC-like protective properties and displays reduced levels in prion infections[J] . EMBO J,2007,26(17):4038-4050.DOI:10.1038/sj.emboj.7601830 [6] Wang HY, Wan JY, Wang W, et al. Overexpression of Shadoo protein in transgenic mice does not impact the pathogenesis of scrapie[J] . Neurosci Lett, 2011, 496(1): 1-4. DOI:10.1016/j.neulet.2011.03.073 [7] Bolton DC, Bendheim PE, Marmorstei AD, et al. Isolation and structural studies of the intact scrapie agent protein[J] . Arch Biochem Biophys,1987, 258(2):579-590. DOI: 10.1016/0003-9861(87)90380-8 [8] Watts JC, Stohr J, Bhardwaj S, et al. Protease-resistant prions selectively decrease Shadoo protein[J] . PLoS Pathog, 2011, 7(11):e1002382. DOI: 10.1371/journal.ppat.1002382 [9] Riemer C, Schultz J, Burwinkel M, et al. Accelerated prion replication in, but prolonged survival times of, prion-infected CXCR3-/- mice[J] . J Virol, 2008, 82(24):12464-12471. DOI: 10.1128/JVI.01371-08