Shadoo/PrP (Sprn^0/0/Prnp^0/0) double knockout mice

References

• Bouché N, Bouchez D. Arabidopsis gene knockout: phenotypes wanted. Curr Opin Plant Biol 2001; 4:111 - 7; http://dx.doi.org/10.1016/S1369-5266(00)00145-X; PMID: 11228432
   PubMed Web of Science ®Google Scholar
• Kim SK. Http://C. elegans: mining the functional genomic landscape. Nat Rev Genet 2001; 2:681 - 9; http://dx.doi.org/10.1038/35088523; PMID: 11533717
   PubMed Web of Science ®Google Scholar
• Barbaric I, Miller G, Dear TN. Appearances can be deceiving: phenotypes of knockout mice. Brief Funct Genomic Proteomic 2007; 6:91 - 103; http://dx.doi.org/10.1093/bfgp/elm008; PMID: 17584761
   PubMedGoogle Scholar
• Watts JC, Westaway D. The prion protein family: diversity, rivalry, and dysfunction. Biochim Biophys Acta 2007; 1772:654 - 72; http://dx.doi.org/10.1016/j.bbadis.2007.05.001; PMID: 17562432
   PubMed Web of Science ®Google Scholar
• Aguzzi A, Sigurdson C, Heikenwaelder M. Molecular mechanisms of prion pathogenesis. Annu Rev Pathol 2008; 3:11 - 40; http://dx.doi.org/10.1146/annurev.pathmechdis.3.121806.154326; PMID: 18233951
   PubMed Web of Science ®Google Scholar
• Linden R, Martins VR, Prado MA, Cammarota M, Izquierdo I, Brentani RR. Physiology of the prion protein. Physiol Rev 2008; 88:673 - 728; http://dx.doi.org/10.1152/physrev.00007.2007; PMID: 18391177
   PubMed Web of Science ®Google Scholar
• Bounhar Y, Zhang Y, Goodyer CG, LeBlanc A. Prion protein protects human neurons against Bax-mediated apoptosis. J Biol Chem 2001; 276:39145 - 9; http://dx.doi.org/10.1074/jbc.C100443200; PMID: 11522774
   PubMed Web of Science ®Google Scholar
• Mehrpour M, Codogno P. Prion protein: From physiology to cancer biology. Cancer Lett 2010; 290:1 - 23; http://dx.doi.org/10.1016/j.canlet.2009.07.009; PMID: 19674833
   PubMed Web of Science ®Google Scholar
• Aguzzi A, Baumann F, Bremer J. The prion’s elusive reason for being. Annu Rev Neurosci 2008; 31:439 - 77; http://dx.doi.org/10.1146/annurev.neuro.31.060407.125620; PMID: 18558863
   PubMed Web of Science ®Google Scholar
• Shmerling D, Hegyi I, Fischer M, Blättler T, Brandner S, Götz J, et al. Expression of amino-terminally truncated PrP in the mouse leading to ataxia and specific cerebellar lesions. Cell 1998; 93:203 - 14; http://dx.doi.org/10.1016/S0092-8674(00)81572-X; PMID: 9568713
   PubMed Web of Science ®Google Scholar
• Premzl M, Sangiorgio L, Strumbo B, Marshall Graves JA, Simonic T, Gready JE. Shadoo, a new protein highly conserved from fish to mammals and with similarity to prion protein. Gene 2003; 314:89 - 102; http://dx.doi.org/10.1016/S0378-1119(03)00707-8; PMID: 14527721
   PubMed Web of Science ®Google Scholar
• Watts JC, Drisaldi B, Ng V, Yang J, Strome B, Horne P, et al. The CNS glycoprotein Shadoo has PrP(C)-like protective properties and displays reduced levels in prion infections. EMBO J 2007; 26:4038 - 50; http://dx.doi.org/10.1038/sj.emboj.7601830; PMID: 17703189
   PubMed Web of Science ®Google Scholar
• Watts JC, Huo H, Bai Y, Ehsani S, Jeon AH, Shi T, et al. Interactome analyses identify ties of PrP and its mammalian paralogs to oligomannosidic N-glycans and endoplasmic reticulum-derived chaperones. PLoS Pathog 2009; 5:e1000608; http://dx.doi.org/10.1371/journal.ppat.1000608; PMID: 19798432
   PubMed Web of Science ®Google Scholar
• Daude N, Wohlgemuth S, Brown R, Pitstick R, Gapeshina H, Yang J, et al. Knockout of the prion protein (PrP)-like Sprn gene does not produce embryonic lethality in combination with PrP(C)-deficiency. Proc Natl Acad Sci U S A 2012; 109:9035 - 40; http://dx.doi.org/10.1073/pnas.1202130109; PMID: 22619325
   PubMed Web of Science ®Google Scholar
• Guillot-Sestier MV, Sunyach C, Druon C, Scarzello S, Checler F. The alpha-secretase-derived N-terminal product of cellular prion, N1, displays neuroprotective function in vitro and in vivo. J Biol Chem 2009; 284:35973 - 86; http://dx.doi.org/10.1074/jbc.M109.051086; PMID: 19850936
   PubMed Web of Science ®Google Scholar
• Taylor DR, Parkin ET, Cocklin SL, Ault JR, Ashcroft AE, Turner AJ, et al. Role of ADAMs in the ectodomain shedding and conformational conversion of the prion protein. J Biol Chem 2009; 284:22590 - 600; http://dx.doi.org/10.1074/jbc.M109.032599; PMID: 19564338
   PubMed Web of Science ®Google Scholar
• Vincent B, Paitel E, Saftig P, Frobert Y, Hartmann D, De Strooper B, et al. The disintegrins ADAM10 and TACE contribute to the constitutive and phorbol ester-regulated normal cleavage of the cellular prion protein. J Biol Chem 2001; 276:37743 - 6; PMID: 11477090
   PubMed Web of Science ®Google Scholar
• Oliveira-Martins JB, Yusa S, Calella AM, Bridel C, Baumann F, Dametto P, et al. Unexpected tolerance of alpha-cleavage of the prion protein to sequence variations. PLoS One 2010; 5:e9107; http://dx.doi.org/10.1371/journal.pone.0009107; PMID: 20161712
   PubMed Web of Science ®Google Scholar
• Vianna CR, Coppari R. A treasure trove of hypothalamic neurocircuitries governing body weight homeostasis. Endocrinology 2011; 152:11 - 8; http://dx.doi.org/10.1210/en.2010-0778; PMID: 21068159
   PubMed Web of Science ®Google Scholar
• Outram GW. Changes in drinking and feeding habits of mice with experimental scrapie. J Comp Pathol 1972; 82:415 - 27; http://dx.doi.org/10.1016/0021-9975(72)90041-2; PMID: 4630696
   PubMed Web of Science ®Google Scholar
• Bailey JD, Berardinelli JG, Rocke TE, Bessen RA. Prominent pancreatic endocrinopathy and altered control of food intake disrupt energy homeostasis in prion diseases. J Endocrinol 2008; 197:251 - 63; http://dx.doi.org/10.1677/JOE-07-0516; PMID: 18434355
   PubMed Web of Science ®Google Scholar
• Beck JA, Campbell TA, Adamson G, Poulter M, Uphill JB, Molou E, et al. Association of a null allele of SPRN with variant Creutzfeldt-Jakob disease. J Med Genet 2008; 45:813 - 7; http://dx.doi.org/10.1136/jmg.2008.061804; PMID: 18805828
   PubMed Web of Science ®Google Scholar
• Westaway D, Genovesi S, Daude N, Brown R, Lau A, Lee I, et al. Down-regulation of Shadoo in prion infections traces a pre-clinical event inversely related to PrP(Sc) accumulation. PLoS Pathog 2011; 7:e1002391; http://dx.doi.org/10.1371/journal.ppat.1002391; PMID: 22114562
   PubMed Web of Science ®Google Scholar
• Watts JC, Stöhr J, Bhardwaj S, Wille H, Oehler A, Dearmond SJ, et al. Protease-resistant prions selectively decrease Shadoo protein. PLoS Pathog 2011; 7:e1002382; http://dx.doi.org/10.1371/journal.ppat.1002382; PMID: 22163178
   PubMed Web of Science ®Google Scholar
• Miyazawa K, Manuelidis L. Agent-specific Shadoo responses in transmissible encephalopathies. J Neuroimmune Pharmacol 2010; 5:155 - 63; http://dx.doi.org/10.1007/s11481-010-9191-1; PMID: 20112073
   PubMed Web of Science ®Google Scholar
• Collinge J, Sidle KC, Meads J, Ironside J, Hill AF. Molecular analysis of prion strain variation and the aetiology of ‘new variant’ CJD. Nature 1996; 383:685 - 90; http://dx.doi.org/10.1038/383685a0; PMID: 8878476
   PubMed Web of Science ®Google Scholar
• Imran M, Mahmood S. An overview of animal prion diseases. Virol J 2011; 8:493; http://dx.doi.org/10.1186/1743-422X-8-493; PMID: 22044871
   PubMed Web of Science ®Google Scholar
• Vilette D. Cell models of prion infection. Vet Res 2008; 39:10; http://dx.doi.org/10.1051/vetres:2007049; PMID: 18073097
   PubMed Web of Science ®Google Scholar
• Young R, Passet B, Vilotte M, Cribiu EP, Béringue V, Le Provost F, et al. The prion or the related Shadoo protein is required for early mouse embryogenesis. FEBS Lett 2009; 583:3296 - 300; http://dx.doi.org/10.1016/j.febslet.2009.09.027; PMID: 19766638
   PubMed Web of Science ®Google Scholar
• Sakaguchi S, Katamine S, Nishida N, Moriuchi R, Shigematsu K, Sugimoto T, et al. Loss of cerebellar Purkinje cells in aged mice homozygous for a disrupted PrP gene. Nature 1996; 380:528 - 31; http://dx.doi.org/10.1038/380528a0; PMID: 8606772
   PubMed Web of Science ®Google Scholar
• Moore RC, Lee IY, Silverman GL, Harrison PM, Strome R, Heinrich C, et al. Ataxia in prion protein (PrP)-deficient mice is associated with upregulation of the novel PrP-like protein doppel. J Mol Biol 1999; 292:797 - 817; http://dx.doi.org/10.1006/jmbi.1999.3108; PMID: 10525406
   PubMed Web of Science ®Google Scholar
• Silverman GL, Qin K, Moore RC, Yang Y, Mastrangelo P, Tremblay P, et al. Doppel is an N-glycosylated, glycosylphosphatidylinositol-anchored protein. Expression in testis and ectopic production in the brains of Prnp(0/0) mice predisposed to Purkinje cell loss. J Biol Chem 2000; 275:26834 - 41; PMID: 10842180
   PubMed Web of Science ®Google Scholar
• Passet B, Young R, Makhzami S, Vilotte M, Jaffrezic F, Halliez S, et al. Prion protein and shadoo are involved in overlapping embryonic pathways and trophoblastic development. PLoS One 2012; 7:e41959; http://dx.doi.org/10.1371/journal.pone.0041959; PMID: 22860039
   PubMedGoogle Scholar
• Katamine S, Nishida N, Sugimoto T, Noda T, Sakaguchi S, Shigematsu K, et al. Impaired motor coordination in mice lacking prion protein. Cell Mol Neurobiol 1998; 18:731 - 42; http://dx.doi.org/10.1023/A:1020234321879; PMID: 9876879
   PubMed Web of Science ®Google Scholar
• Bremer J, Baumann F, Tiberi C, Wessig C, Fischer H, Schwarz P, et al. Axonal prion protein is required for peripheral myelin maintenance. Nat Neurosci 2010; 13:310 - 8; http://dx.doi.org/10.1038/nn.2483; PMID: 20098419
   PubMed Web of Science ®Google Scholar
• Schmitt-Ulms G, Ehsani S, Watts JC, Westaway D, Wille H. Evolutionary descent of prion genes from the ZIP family of metal ion transporters. PLoS One 2009; 4:e7208; http://dx.doi.org/10.1371/journal.pone.0007208; PMID: 19784368
   PubMedGoogle Scholar
• Li A, Christensen HM, Stewart LR, Roth KA, Chiesa R, Harris DA. Neonatal lethality in transgenic mice expressing prion protein with a deletion of residues 105-125. EMBO J 2007; 26:548 - 58; http://dx.doi.org/10.1038/sj.emboj.7601507; PMID: 17245437
   PubMed Web of Science ®Google Scholar
• Polazzi E, Monti B. Microglia and neuroprotection: from in vitro studies to therapeutic applications. Prog Neurobiol 2010; 92:293 - 315; http://dx.doi.org/10.1016/j.pneurobio.2010.06.009; PMID: 20609379
   PubMed Web of Science ®Google Scholar