January 22, 2018



  1. Aguilar-Calvo P, Sevillano AM, Rasool S, Cao KJ, Randolph LM, Rissman RA, et al. Noninvasive Antemortem Detection of Retinal Prions by a Fluorescent Tracer. J Alzheimers Dis. 2022;88(3):1137-45.
  2. Cordano C, Nourbakhsh B, Yiu HH, Papinutto N, Caverzasi E, Abdelhak AC, et al. Differences in Age-Related Retinal and Cortical Atrophy Rates in Multiple Sclerosis. Neurology. 2022.
  3. Gonias SL, Banki MA, Azmoon P, Romero HK, Sigurdson CJ, Mantuano E, et al. Cellular prion protein in human plasma-derived extracellular vesicles promotes neurite outgrowth via the NMDA receptor-LRP1 receptor system. J Biol Chem. 2022;298(3):101642.
  4. Goodwill VS, Dryden I, Choi J, De Lillo C, Soldau K, Llibre-Guerra J, et al. Minimal change prion retinopathy: Morphometric comparison of retinal and brain prion deposits in Creutzfeldt-Jakob disease. Exp Eye Res. 2022;222:109172.
  5. Mantuano E, Azmoon P, Banki MA, Sigurdson CJ, Campana WM, Gonias SL. A Soluble PrP(C) Derivative and Membrane-Anchored PrP(C) in Extracellular Vesicles Attenuate Innate Immunity by Engaging the NMDA-R/LRP1 Receptor Complex. J Immunol. 2022;208(1):85-96.
  6. Ojeda-Juárez D, Lawrence JA, Soldau K, Pizzo DP, Wheeler E, Aguilar-Calvo P, et al. Prions induce an early Arc response and a subsequent reduction in mGluR5 in the hippocampus. Neurobiol Dis. 2022;172:105834.
  7. Aguilar-Calvo P, Callender JA, Sigurdson CJ. Short and sweet: How glycans impact prion conversion, cofactor interactions, and cross-species transmission. PLoS Pathog. 2021;17(1):e1009123.
  8. Cao KJ, Kim JH, Kroeger H, Gaffney PM, Lin JH, Sigurdson CJ, et al. ARCAM-1 Facilitates Fluorescence Detection of Amyloid-Containing Deposits in the Retina. Translational vision science & technology. 2021;10(7):5.
  9. Liu H, Kim C, Haldiman T, Sigurdson CJ, Nyström S, Nilsson KPR, et al. Distinct conformers of amyloid beta accumulate in the neocortex of patients with rapidly progressive Alzheimer’s disease. J Biol Chem. 2021;297(5):101267.
  10. Meisl G, Kurt T, Condado-Morales I, Bett C, Sorce S, Nuvolone M, et al. Scaling analysis reveals the mechanism and rates of prion replication in vivo. Nat Struct Mol Biol. 2021;28(4):365-72.


    1. Aguilar-Calvo P, Sevillano AM, Bapat J, Soldau K, Sandoval DR, Altmeppen HC, et al. Shortening heparan sulfate chains prolongs survival and reduces parenchymal plaques in prion disease caused by mobile, ADAM10-cleaved prions. Acta Neuropathol. 2020;139(3):527-46.
    2. Asher DM, Belay E, Bigio E, Brandner S, Brubaker SA, Caughey B, et al. Risk of Transmissibility From Neurodegenerative Disease-Associated Proteins: Experimental Knowns and Unknowns. J Neuropathol Exp Neurol. 2020;79(11):1141-6.
    3. Callender JA, Sevillano AM, Soldau K, Kurt TD, Schumann T, Pizzo DP, et al. Prion protein post-translational modifications modulate heparan sulfate binding and limit aggregate size in prion disease. Neurobiol Dis. 2020;142:104955.
    4. Mantuano E, Azmoon P, Banki MA, Lam MS, Sigurdson CJ, and Gonias SL. A soluble derivative of PrP(C) activates cell-signaling and regulates cell physiology through LRP1 and the NMDA receptor. J Biol Chem. 2020;295(41):14178-88.
    5. Sevillano AM, Aguilar-Calvo P, Kurt TD, Lawrence JA, Soldau K, Nam TH, et al. Prion protein glycans reduce intracerebral fibril formation and spongiosis in prion disease. J Clin Invest. 2020;130(3):1350-62.
    6. Sigurdson CJ, Bartz JC, and Glatzel M. Cellular and Molecular Mechanisms of Prion Disease. Annu Rev Pathol. 2019;14:497-516.
    7. Glatzel M, and Sigurdson CJ. Recent advances on the molecular pathogenesis of prion diseasesBrain Pathol. 2019;29(2):245-7.
    8. Kollmer M, Close W, Funk L, Rasmussen J, Bsoul A, Schierhorn A, et al. Cryo-EM structure and polymorphism of Aβ amyloid fibrils purified from Alzheimer’s brain tissue. Nat Commun. 2019;10(1):4760.
    9. Kraus A, Saijo E, Metrick MA, 2nd, Newell K, Sigurdson CJ, Zanusso G, et al. Seeding selectivity and ultrasensitive detection of tau aggregate conformers of Alzheimer disease. Acta Neuropathol. 2019;137(4):585-98.
    10.  Glatzel M, Sigurdson CJ. Recent advances on the molecular pathogenesis of prion diseases. Brain Pathol. 29: 245-247.2019;bpa.12693.
    11. Kollmer M, Close W, Funk L, Rasmussen J, Bsoul A, Schierhorn A, Schmidt M, Sigurdson CJ, Jucker M, Fandrich M. Cryo-EM structure and polymorphism of Abeta amyloid fibrils purified from Alzheimer’s brain tissue. Nat Commun. 10.2019 4760.2019;s41467-019-12683-8
    12. Kraus A, Saijo E, Metrick MA, 2nd, Newell K, Sigurdson CJ, Zanusso G, Ghetti B, Caughey B. Seeding selectivity and ultrasensitive detection of tau aggregate conformers of Alzheimer disease. Acta Neuropathol. 137.2019 585-598.2019;s00401-018-1947-3
    13. Sigurdson CJ, Bartz JC, Glatzel M Cellular and Molecular Mechanisms of Prion Disease. Annu Rev Pathol. 14: 497-516.2019.
    14. Orru CD, Soldau K, Cordano C, Llibre-Guerra J, Green AJ, Sanchez H, Groveman BR, Edland SD, Safar JG, Lin JHet al. Prion Seeds Distribute throughout the Eyes of Sporadic Creutzfeldt-Jakob Disease Patients. mBio. 9.2018;mBio.02095-18
    15. Cao KJ, Elbel KM, Cifelli JL, Cirera J, Sigurdson CJ, Paesani F, et al. Solvation-Guided Design of Fluorescent Probes for Discrimination of Amyloids. Sci Rep. 2018;8(1):695.
    16. Aguilar-Calvo P, Bett C, Sevillano AM, Kurt TD, Lawrence J, Soldau K, Hammarstrom P, Nilsson KPR, Sigurdson CJ. Generation of novel neuroinvasive prions following intravenous challenge Brain Pathol. 2018.
    17. Cao KJ, Elbel KM, Cifelli JL, Cirera J, Sigurdson CJ, Paesani F, Theodorakis EA, Yang J. Solvation-Guided Design of Fluorescent Probes for Discrimination of Amyloids. Sci Rep.2018; 8:6950.
    18. Orrù CD, Soldau K, Cordano C, Llibre-Guerra J, Green AJ, Sanchez H, et al. Prion Seeds Distribute throughout the Eyes of Sporadic Creutzfeldt-Jakob Disease Patients. mBio. 2018;9(6).
    19. Aguilar-Calvo P, Bett C, Sevillano AM, Kurt TD, Lawrence J, Soldau K, et al. Generation of novel neuroinvasive prions following intravenous challenge. Brain Pathol. 2018;28(6):999-1011.
    20. Sikorska B, Gajos A, Bogucki A, Zielonka E, Sigurdson C, and Liberski PP. Electron microscopic and confocal laser microscopy analysis of amyloid plaques in chronic wasting disease transmitted to transgenic mice. Prion. 2017;11(6):431-9.
    21. Sikorska B, Gajos A, Bogucki A, Zielonka E, Sigurdson C, and Liberski PP. Electron microscopic and confocal laser microscopy analysis of amyloid plaques in chronic wasting disease transmitted to transgenic mice. Prion. 2017;11(6):431-9.
    22. Kurt TD, Aguilar-Calvo P, Jiang L, Rodriguez JA, Alderson N, Eisenberg DS, and Sigurdson CJ. Asparagine and glutamine ladders promote cross-species prion conversion. J Biol Chem. 2017.
    23. Bett C, Lawrence J, Kurt TD, Orru C, Aguilar-Calvo P, Kincaid AE, Surewicz WK, Caughey B, Wu C, and Sigurdson CJ. Enhanced neuroinvasion by smaller, soluble prions. Acta neuropathologica communications. 2017;5(1):32.
    24. Aguilar-Calvo P, Xiao X, Bett C, Erana H, Soldau K, Castilla J, Nilsson KP, Surewicz WK, and Sigurdson CJ. Post-translational modifications in PrP expand the conformational diversity of prions in vivo. Sci Rep. 2017;7(43):295.
    25. Kurt TD, and Sigurdson CJ. Cross-species transmission of CWD prions. Prion. 2016;10(1):83-91.
    26. Klionsky DJ, Abdelmohsen K, Abe A, Abedin MJ, Abeliovich H, Acevedo Arozena A, Adachi H, Adams CM, Adams PD, Adeli K, et al. Guidelines for the use and interpretation of assays for monitoring autophagy (3rd edition). Autophagy. 2016;12(1):1-222.
    27. Keene CD, Darvas M, Kraemer B, Liggitt D, Sigurdson C, and Ladiges W. Neuropathological assessment and validation of mouse models for Alzheimer’s disease: applying NIA-AA guidelines. Pathobiology of aging & age related diseases. 2016;6(32397).
    28. Gaffney PM, Witte C, Clifford DL, Imai DM, O’Brien TD, Trejo M, Liberta F, Annamalai K, Fandrich M, Masliah E, et al. Systemic Amyloid A Amyloidosis in Island Foxes (Urocyon littoralis): Severity and Risk Factors. Vet Pathol. 2016;53(3):637-47.
    29. Annamalai K, Guhrs KH, Koehler R, Schmidt M, Michel H, Loos C, Gaffney PM, Sigurdson CJ, Hegenbart U, Schonland S, et al. Polymorphism of Amyloid Fibrils In Vivo. Angew Chem Int Ed Engl. 2016;55(15):4822-5.
    30. Shirani H, Linares M, Sigurdson CJ, Lindgren M, Norman P, and Nilsson KP. A Palette of Fluorescent Thiophene-Based Ligands for the Identification of Protein Aggregates. Chemistry (Easton). 2015;21(43):15133-7.
    31. Kurt TD, Jiang L, Fernandez-Borges N, Bett C, Liu J, Yang T, Spraker TR, Castilla J, Eisenberg D, Kong Q, et al. Human prion protein sequence elements impede cross-species chronic wasting disease transmission. J Clin Invest. 2015;125(6):2548.
    32. Hiramatsu N, Chiang WC, Kurt TD, Sigurdson CJ, and Lin JH. Multiple Mechanisms of Unfolded Protein Response-Induced Cell Death. Am J Pathol. 2015;185(7):1800-8.
    33. Gaffney PM, Barr B, Rowe JD, Bett C, Drygiannakis I, Giannitti F, Trejo M, Ghassemian M, Martin P, Masliah E, et al. Protein profiling of isolated uterine AA amyloidosis causing fetal death in goats. FASEB J. 2015;29(3):911-9.


    1. Magnusson K, Simon R, Sjolander D, Sigurdson CJ, Hammarstrom P, and Nilsson KP. Multimodal fluorescence microscopy of prion strain specific PrP deposits stained by thiophene-based amyloid ligands. Prion. 2014;8(4):319-29.
    2. Kurt TD, Bett C, Fernandez-Borges N, Joshi-Barr S, Hornemann S, Rulicke T, Castilla J, Wuthrich K, Aguzzi A, and Sigurdson CJ. Prion transmission prevented by modifying the beta2-alpha2 loop structure of host PrPC. J Neurosci. 2014;34(3):1022-7.
    3. Joshi-Barr S, Bett C, Chiang WC, Trejo M, Goebel HH, Sikorska B, Liberski P, Raeber A, Lin JH, Masliah E, et al. De novo prion aggregates trigger autophagy in skeletal muscle. J Virol. 2014;88(4):2071-82.
    4. Gaffney PM, Imai DM, Clifford DL, Ghassemian M, Sasik R, Chang AN, O’Brien TD, Coppinger J, Trejo M, Masliah E, et al. Proteomic analysis of highly prevalent amyloid A amyloidosis endemic to endangered island foxes. PLoS One. 2014;9(11):e113765.
    5. Perrott MR, Sigurdson CJ, Mason GL, and Hoover EA. Mucosal transmission and pathogenesis of chronic wasting disease in ferrets. J Gen Virol. 2013;94(Pt 2):432-42.
    6. Bett C, Kurt TD, Lucero M, Trejo M, Rozemuller AJ, Kong Q, Nilsson KP, Masliah E, Oldstone MB, and Sigurdson CJ. Defining the conformational features of anchorless, poorly neuroinvasive prions. PLoS Pathog. 2013;9(4):e1003280.
    7. Sajnani G, Silva CJ, Ramos A, Pastrana MA, Onisko BC, Erickson ML, Antaki EM, Dynin I, Vazquez-Fernandez E, Sigurdson CJ, et al. PK-sensitive PrP is infectious and shares basic structural features with PK-resistant PrP. PLoS Pathog. 2012;8(3):e1002547.
    8. Perrott MR, Sigurdson CJ, Mason GL, and Hoover EA. Evidence for distinct chronic wasting disease (CWD) strains in experimental CWD in ferrets. J Gen Virol. 2012;93(Pt 1):212-21.
    9. Mitchell GB, Sigurdson CJ, O’Rourke KI, Algire J, Harrington NP, Walther I, Spraker TR, and Balachandran A. Experimental oral transmission of chronic wasting disease to reindeer (Rangifer tarandus tarandus). PLoS One. 2012;7(6):e39055.
    10. Masliah E, Rockenstein E, Inglis C, Adame A, Bett C, Lucero M, and Sigurdson CJ. Prion infection promotes extensive accumulation of alpha-synuclein in aged human alpha-synuclein transgenic mice. Prion. 2012;6(2):184-90.
    11. Cao K, Farahi M, Dakanali M, Chang WM, Sigurdson CJ, Theodorakis EA, and Yang J. Aminonaphthalene 2-cyanoacrylate (ANCA) probes fluorescently discriminate between amyloid-beta and prion plaques in brain. J Am Chem Soc. 2012;134(42):17338-41.
    12. Bett C, Joshi-Barr S, Lucero M, Trejo M, Liberski P, Kelly JW, Masliah E, and Sigurdson CJ. Biochemical properties of highly neuroinvasive prion strains. PLoS Pathog. 2012;8(2):e1002522.
    13. Bett C, Fernandez-Borges N, Kurt TD, Lucero M, Nilsson KP, Castilla J, and Sigurdson CJ. Structure of the beta2-alpha2 loop and interspecies prion transmission. FASEB J. 2012;26(7):2868-76.
    14. Sigurdson CJ, Joshi-Barr S, Bett C, Winson O, Manco G, Schwarz P, Rulicke T, Nilsson KP, Margalith I, Raeber A, et al. Spongiform encephalopathy in transgenic mice expressing a point mutation in the beta2-alpha2 loop of the prion protein. J Neurosci. 2011;31(39):13840-7.
    15. Sigurdson CJ, Bartz JC, and Nilsson KP. Tracking protein aggregate interactions. Prion. 2011;5(2):52-5.
    16. Ligios C, Cancedda MG, Carta A, Santucciu C, Maestrale C, Demontis F, Saba M, Patta C, DeMartini JC, Aguzzi A, et al. Sheep with scrapie and mastitis transmit infectious prions through the milk. J Virol. 2011;85(2):1136-9.
    17. Chang WM, Dakanali M, Capule CC, Sigurdson CJ, Yang J, and Theodorakis EA. ANCA: A Family of Fluorescent Probes that Bind and Stain Amyloid Plaques in Human Tissue. ACS Chem Neurosci. 2011;2(5):249-55.
    18. Wegrzyn JL, Bark SJ, Funkelstein L, Mosier C, Yap A, Kazemi-Esfarjani P, La Spada AR, Sigurdson C, O’Connor DT, and Hook V. Proteomics of dense core secretory vesicles reveal distinct protein categories for secretion of neuroeffectors for cell-cell communication. J Proteome Res. 2010;9(10):5002-24.
    19. Sigurdson CJ, Nilsson KP, Hornemann S, Manco G, Fernandez-Borges N, Schwarz P, Castilla J, Wuthrich K, and Aguzzi A. A molecular switch controls interspecies prion disease transmission in mice. J Clin Invest. 2010;120(7):2590-9.
    20. Sandberg MK, Al-Doujaily H, Sigurdson CJ, Glatzel M, O’Malley C, Powell C, Asante EA, Linehan JM, Brandner S, Wadsworth JD, et al. Chronic wasting disease prions are not transmissible to transgenic mice overexpressing human prion protein. J Gen Virol. 2010;91(Pt 10):2651-7.
    21. Nilsson KP, Joshi-Barr S, Winson O, and Sigurdson CJ. Prion strain interactions are highly selective. J Neurosci. 2010;30(36):12094-102.
    22. Lee SJ, Desplats P, Sigurdson C, Tsigelny I, and Masliah E. Cell-to-cell transmission of non-prion protein aggregates. Nat Rev Neurol. 2010;6(12):702-6.


    1. Sigurdson CJ, Nilsson KP, Hornemann S, Heikenwalder M, Manco G, Schwarz P, Ott D, Rulicke T, Liberski PP, Julius C, et al. De novo generation of a transmissible spongiform encephalopathy by mouse transgenesis. Proc Natl Acad Sci U S A. 2009;106(1):304-9.
    2. Sigurdson CJ, Heikenwalder M, Manco G, Barthel M, Schwarz P, Stecher B, Krautler NJ, Hardt WD, Seifert B, MacPherson AJ, et al. Bacterial Colitis Increases Susceptibility to Oral Prion Disease. J Infect Dis. 2009;199(2):243-52.
    3. Aslund A, Sigurdson CJ, Klingstedt T, Grathwohl S, Bolmont T, Dickstein DL, Glimsdal E, Prokop S, Lindgren M, Konradsson P, et al. Novel pentameric thiophene derivatives for in vitro and in vivo optical imaging of a plethora of protein aggregates in cerebral amyloidoses. ACS Chem Biol. 2009;4(8):673-84.
    4. Sigurdson CJ, Mathiason CK, Perrott MR, Eliason GA, Spraker TR, Glatzel M, Manco G, Bartz JC, Miller MW, and Hoover EA. Experimental chronic wasting disease (CWD) in the ferret. J Comp Pathol. 2008;138(4):189-96.
    5. Sigurdson CJ. A prion disease of cervids: chronic wasting disease. Vet Res. 2008;39(4):41.
    6. Polymenidou M, Moos R, Scott M, Sigurdson C, Shi YZ, Yajima B, Hafner-Bratkovic I, Jerala R, Hornemann S, Wuthrich K, et al. The POM monoclonals: a comprehensive set of antibodies to non-overlapping prion protein epitopes. PLoS One. 2008;3(12):e3872.
    7. Heikenwalder M, Kurrer MO, Margalith I, Kranich J, Zeller N, Haybaeck J, Polymenidou M, Matter M, Bremer J, Jackson WS, et al. Lymphotoxin-dependent prion replication in inflammatory stromal cells of granulomas. Immunity. 2008;29(6):998-1008.
    8. Aguzzi A, Sigurdson C, and Heikenwaelder M. Molecular mechanisms of prion pathogenesis. Annu Rev Pathol. 2008;3(11-40).
    9. Sigurdson CJ, Nilsson KP, Hornemann S, Manco G, Polymenidou M, Schwarz P, Leclerc M, Hammarstrom P, Wuthrich K, and Aguzzi A. Prion strain discrimination using luminescent conjugated polymers. Nat Methods. 2007;4(12):1023-30.
    10. Sigurdson CJ, and Aguzzi A. Chronic wasting disease. Biochim Biophys Acta. 2007;1772(6):610-8.
    11. Sigurdson CJ, Manco G, Schwarz P, Liberski P, Hoover EA, Hornemann S, Polymenidou M, Miller MW, Glatzel M, and Aguzzi A. Strain fidelity of chronic wasting disease upon murine adaptation. J Virol. 2006;80(24):12303-11.
    12. Mathiason CK, Powers JG, Dahmes SJ, Osborn DA, Miller KV, Warren RJ, Mason GL, Hays SA, Hayes-Klug J, Seelig DM, et al. Infectious prions in the saliva and blood of deer with chronic wasting disease. Science. 2006;314(5796):133-6.
    13. Angers RC, Browning SR, Seward TS, Sigurdson CJ, Miller MW, Hoover EA, and Telling GC. Prions in skeletal muscles of deer with chronic wasting disease. Science. 2006;311(5764):1117.
    14. Sigurdson C, Polymenidou M, and Aguzzi A. Reconstructing prions: fibril assembly from simple yeast to complex mammals. Neurodegener Dis. 2005;2(1):1-5.
    15. Ligios C, Sigurdson CJ, Santucciu C, Carcassola G, Manco G, Basagni M, Maestrale C, Cancedda MG, Madau L, and Aguzzi A. PrPSc in mammary glands of sheep affected by scrapie and mastitis. Nat Med. 2005;11(11):1137-8.


    1. Heikenwalder M, Polymenidou M, Junt T, Sigurdson C, Wagner H, Akira S, Zinkernagel R, and Aguzzi A. Lymphoid follicle destruction and immunosuppression after repeated CpG oligodeoxynucleotide administration. Nat Med. 2004;10(2):187-92.
    2. Aguzzi A, and Sigurdson CJ. Antiprion immunotherapy: to suppress or to stimulate? Nat Rev Immunol. 2004;4(9):725-36.
    3. Sigurdson CJ, and Miller MW. Other animal prion diseases. Br Med Bull. 2003;66(199-212).
    4. Spraker TR, Zink RR, Cummings BA, Sigurdson CJ, Miller MW, and O’Rourke KI. Distribution of protease-resistant prion protein and spongiform encephalopathy in free-ranging mule deer (Odocoileus hemionus) with chronic wasting disease. Vet Pathol. 2002;39(5):546-56.
    5. Sigurdson CJ, Basaraba RJ, Mazzaferro EM, and Gould DH. Globoid cell-like leukodystrophy in a domestic longhaired cat. Vet Pathol. 2002;39(4):494-6.
    6. Sigurdson CJ, Barillas-Mury C, Miller MW, Oesch B, van Keulen LJ, Langeveld JP, and Hoover EA. PrP(CWD) lymphoid cell targets in early and advanced chronic wasting disease of mule deer. J Gen Virol. 2002;83(Pt 10):2617-28.
    7. Sigurdson CJ, Spraker TR, Miller MW, Oesch B, and Hoover EA. PrP(CWD) in the myenteric plexus, vagosympathetic trunk and endocrine glands of deer with chronic wasting disease. J Gen Virol. 2001;82(Pt 10):2327-34.
    8. Sigurdson CJ, Williams ES, Miller MW, Spraker TR, O’Rourke KI, and Hoover EA. Oral transmission and early lymphoid tropism of chronic wasting disease PrPres in mule deer fawns (Odocoileus hemionus). J Gen Virol. 1999;80 ( Pt 10)(2757-64).
    9. Loneragan GH, Gould DH, Callan RJ, Sigurdson CJ, and Hamar DW. Association of excess sulfur intake and an increase in hydrogen sulfide concentrations in the ruminal gas cap of recently weaned beef calves with polioencephalomalacia. J Am Vet Med Assoc. 1998;213(11):1599-604, 71.