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Age at onset in genetic prion disease and the design of preventive clinical trials.

Minikel EV, Vallabh SM, Orseth MC, Brandel JP, Haïk S, Laplanche JL, Zerr I, Parchi P, Capellari S, Safar J, Kenny J, Fong JC, Takada LT, Ponto C, Hermann P, Knipper T, Stehmann C, Kitamoto T, Ae R, Hamaguchi T, Sanjo N, Tsukamoto T, Mizusawa H, Collins SJ, Chiesa R, Roiter I, de Pedro-Cuesta J, Calero M, Geschwind MD, Yamada M, Nakamura Y, Mead S.

Neurology. 2019 Jul 9;93(2):e125-e134. doi: 10.1212/WNL.0000000000007745. Epub 2019 Jun 6.


Early existence and biochemical evolution characterise acutely synaptotoxic PrPSc.

Foliaki ST, Lewis V, Islam AMT, Ellett LJ, Senesi M, Finkelstein DI, Roberts B, Lawson VA, Adlard PA, Collins SJ.

PLoS Pathog. 2019 Apr 10;15(4):e1007712. doi: 10.1371/journal.ppat.1007712. eCollection 2019 Apr.


ATN profiles among cognitively normal individuals and longitudinal cognitive outcomes.

Soldan A, Pettigrew C, Fagan AM, Schindler SE, Moghekar A, Fowler C, Li QX, Collins SJ, Carlsson C, Asthana S, Masters CL, Johnson S, Morris JC, Albert M, Gross AL.

Neurology. 2019 Apr 2;92(14):e1567-e1579. doi: 10.1212/WNL.0000000000007248. Epub 2019 Mar 6.


Secreted cellular prion protein binds doxorubicin and correlates with anthracycline resistance in breast cancer.

Wiegmans AP, Saunus JM, Ham S, Lobb R, Kutasovic JR, Dalley AJ, Miranda M, Atkinson C, Foliaki ST, Ferguson K, Niland C, Johnstone CN, Lewis V, Collins SJ, Lakhani SR, Al-Ejeh F, Möller A.

JCI Insight. 2019 Feb 26;5. pii: 124092. doi: 10.1172/jci.insight.124092.


Comparative Utility of Manual versus Automated Segmentation of Hippocampus and Entorhinal Cortex Volumes in a Memory Clinic Sample.

Fung YL, Ng KET, Vogrin SJ, Meade C, Ngo M, Collins SJ, Bowden SC.

J Alzheimers Dis. 2019;68(1):159-171. doi: 10.3233/JAD-181172.


Creutzfeldt-Jakob disease surveillance in Australia: update to December 2016.

Klug GM, Sarros S, Stehmann C, Simpson M, McLean C, Masters CL, Collins SJ.

Commun Dis Intell (2018). 2018;42. pii: S2209-6051(18)00023-4. Epub 2018 Dec 17.


Life in the slow drain: Landscape structure affects farm ditch water quality.

Collins SJ, Bellingham L, Mitchell GW, Fahrig L.

Sci Total Environ. 2019 Mar 15;656:1157-1167. doi: 10.1016/j.scitotenv.2018.11.400. Epub 2018 Nov 28.


Prion acute synaptotoxicity is largely driven by protease-resistant PrPSc species.

Foliaki ST, Lewis V, Finkelstein DI, Lawson V, Coleman HA, Senesi M, Islam AMT, Chen F, Sarros S, Roberts B, Adlard PA, Collins SJ.

PLoS Pathog. 2018 Aug 8;14(8):e1007214. doi: 10.1371/journal.ppat.1007214. eCollection 2018 Aug.


Cerebrospinal Fluid Total Prion Protein in the Spectrum of Prion Diseases.

Villar-Piqué A, Schmitz M, Lachmann I, Karch A, Calero O, Stehmann C, Sarros S, Ladogana A, Poleggi A, Santana I, Ferrer I, Mitrova E, Žáková D, Pocchiari M, Baldeiras I, Calero M, Collins SJ, Geschwind MD, Sánchez-Valle R, Zerr I, Llorens F.

Mol Neurobiol. 2019 Apr;56(4):2811-2821. doi: 10.1007/s12035-018-1251-1. Epub 2018 Jul 30.


Prion protein cleavage fragments regulate adult neural stem cell quiescence through redox modulation of mitochondrial fission and SOD2 expression.

Collins SJ, Tumpach C, Groveman BR, Drew SC, Haigh CL.

Cell Mol Life Sci. 2018 Sep;75(17):3231-3249. doi: 10.1007/s00018-018-2790-3. Epub 2018 Mar 24.


The Keepers: The stunning illumination of a cover up of child sexual abuse and institutional betrayal.

Brand BL, Collins SJ, McEwen LE.

J Trauma Dissociation. 2018 Oct-Dec;19(5):607-611. doi: 10.1080/15299732.2018.1452526. Epub 2018 Mar 20. No abstract available.


CSF Tau supplements 14-3-3 protein detection for sporadic Creutzfeldt-Jakob disease diagnosis while transitioning to next generation diagnostics.

Li QX, Varghese S, Sarros S, Stehmann C, Doecke JD, Fowler CJ, Masters CL, Collins SJ.

J Clin Neurosci. 2018 Apr;50:292-293. doi: 10.1016/j.jocn.2018.01.064.


Acute Neurotoxicity Models of Prion Disease.

T Islam AM, Adlard PA, Finkelstein DI, Lewis V, Biggi S, Biasini E, Collins SJ.

ACS Chem Neurosci. 2018 Mar 21;9(3):431-445. doi: 10.1021/acschemneuro.7b00517. Epub 2018 Feb 14. Review.


Iatrogenic Creutzfeldt-Jakob disease with Amyloid-β pathology: an international study.

Cali I, Cohen ML, Haik S, Parchi P, Giaccone G, Collins SJ, Kofskey D, Wang H, McLean CA, Brandel JP, Privat N, Sazdovitch V, Duyckaerts C, Kitamoto T, Belay ED, Maddox RA, Tagliavini F, Pocchiari M, Leschek E, Appleby BS, Safar JG, Schonberger LB, Gambetti P.

Acta Neuropathol Commun. 2018 Jan 8;6(1):5. doi: 10.1186/s40478-017-0503-z.


Molecular Subtyping of PrPres in Human Sporadic CJD Brain Tissue.

Klug GM, Lewis V, Collins SJ.

Methods Mol Biol. 2017;1658:347-354. doi: 10.1007/978-1-4939-7244-9_23.


Prion Diseases.

Whitechurch BC, Welton JM, Collins SJ, Lawson VA.

Adv Neurobiol. 2017;15:335-364. doi: 10.1007/978-3-319-57193-5_13. Review.


In vivo prion models and the disconnection between transmissibility and neurotoxicity.

Senesi M, Lewis V, Kim JH, Adlard PA, Finkelstein DI, Collins SJ.

Ageing Res Rev. 2017 Jul;36:156-164. doi: 10.1016/j.arr.2017.03.007. Epub 2017 Apr 24. Review.


Creutzfeldt-Jakob disease surveillance in Australia: update to December 2015.

Klug GM, Boyd A, Sarros S, Stehmann C, Simpson M, McLean CA, Masters CL, Collins SJ.

Commun Dis Intell Q Rep. 2016 Sep 30;40(3):E368-E376.


Oxidation of Iron under Physiologically Relevant Conditions in Biological Fluids from Healthy and Alzheimer's Disease Subjects.

Lam LQ, Wong BX, Frugier T, Li QX, Collins SJ, Bush AI, Crack PJ, Duce JA.

ACS Chem Neurosci. 2017 Apr 19;8(4):731-736. doi: 10.1021/acschemneuro.6b00411. Epub 2017 Jan 3.


Simplified Murine 3D Neuronal Cultures for Investigating Neuronal Activity and Neurodegeneration.

Collins SJ, Haigh CL.

Cell Biochem Biophys. 2017 Mar;75(1):3-13. doi: 10.1007/s12013-016-0768-z. Epub 2016 Oct 28.


Creutzfeldt-Jakob disease surveillance in Australia: update to December 2014.

Klug GM, Boyd A, Sarros S, Stehmann C, Simpson M, McLean C, Masters CL, Collins SJ.

Commun Dis Intell Q Rep. 2016 Jun 30;40(2):E207-15.


A 2-Substituted 8-Hydroxyquinoline Stimulates Neural Stem Cell Proliferation by Modulating ROS Signalling.

Haigh CL, Tumpach C, Collins SJ, Drew SC.

Cell Biochem Biophys. 2016 Sep;74(3):297-306. doi: 10.1007/s12013-016-0747-4. Epub 2016 Jun 21.


Endoproteolytic cleavage as a molecular switch regulating and diversifying prion protein function.

Haigh CL, Collins SJ.

Neural Regen Res. 2016 Feb;11(2):238-9. doi: 10.4103/1673-5374.177726. No abstract available.


Quantifying prion disease penetrance using large population control cohorts.

Minikel EV, Vallabh SM, Lek M, Estrada K, Samocha KE, Sathirapongsasuti JF, McLean CY, Tung JY, Yu LP, Gambetti P, Blevins J, Zhang S, Cohen Y, Chen W, Yamada M, Hamaguchi T, Sanjo N, Mizusawa H, Nakamura Y, Kitamoto T, Collins SJ, Boyd A, Will RG, Knight R, Ponto C, Zerr I, Kraus TF, Eigenbrod S, Giese A, Calero M, de Pedro-Cuesta J, Haïk S, Laplanche JL, Bouaziz-Amar E, Brandel JP, Capellari S, Parchi P, Poleggi A, Ladogana A, O'Donnell-Luria AH, Karczewski KJ, Marshall JL, Boehnke M, Laakso M, Mohlke KL, Kähler A, Chambert K, McCarroll S, Sullivan PF, Hultman CM, Purcell SM, Sklar P, van der Lee SJ, Rozemuller A, Jansen C, Hofman A, Kraaij R, van Rooij JG, Ikram MA, Uitterlinden AG, van Duijn CM; Exome Aggregation Consortium (ExAC), Daly MJ, MacArthur DG.

Sci Transl Med. 2016 Jan 20;8(322):322ra9. doi: 10.1126/scitranslmed.aad5169.


Prion protein "gamma-cleavage": characterizing a novel endoproteolytic processing event.

Lewis V, Johanssen VA, Crouch PJ, Klug GM, Hooper NM, Collins SJ.

Cell Mol Life Sci. 2016 Feb;73(3):667-83. doi: 10.1007/s00018-015-2022-z. Epub 2015 Aug 23.


The Prion Protein N1 and N2 Cleavage Fragments Bind to Phosphatidylserine and Phosphatidic Acid; Relevance to Stress-Protection Responses.

Haigh CL, Tumpach C, Drew SC, Collins SJ.

PLoS One. 2015 Aug 7;10(8):e0134680. doi: 10.1371/journal.pone.0134680. eCollection 2015.


A genome wide association study links glutamate receptor pathway to sporadic Creutzfeldt-Jakob disease risk.

Sanchez-Juan P, Bishop MT, Kovacs GG, Calero M, Aulchenko YS, Ladogana A, Boyd A, Lewis V, Ponto C, Calero O, Poleggi A, Carracedo Á, van der Lee SJ, Ströbel T, Rivadeneira F, Hofman A, Haïk S, Combarros O, Berciano J, Uitterlinden AG, Collins SJ, Budka H, Brandel JP, Laplanche JL, Pocchiari M, Zerr I, Knight RS, Will RG, van Duijn CM.

PLoS One. 2015 Apr 28;10(4):e0123654. doi: 10.1371/journal.pone.0123654. eCollection 2014.


The prion protein regulates beta-amyloid-mediated self-renewal of neural stem cells in vitro.

Collins SJ, Tumpach C, Li QX, Lewis V, Ryan TM, Roberts B, Drew SC, Lawson VA, Haigh CL.

Stem Cell Res Ther. 2015 Apr 11;6:60. doi: 10.1186/s13287-015-0067-4.


Glycosaminoglycan sulfation determines the biochemical properties of prion protein aggregates.

Ellett LJ, Coleman BM, Shambrook MC, Johanssen VA, Collins SJ, Masters CL, Hill AF, Lawson VA.

Glycobiology. 2015 Jul;25(7):745-55. doi: 10.1093/glycob/cwv014. Epub 2015 Feb 20.


Creutzfeldt-Jakob disease surveillance in Australia, update to December 2013.

Klug GM, Boyd A, Sarros S, Stehmann C, Simpson M, McLean CA, Masters CL, Collins SJ.

Commun Dis Intell Q Rep. 2014 Dec 31;38(4):E348-55.


Neutron reflectometry studies define prion protein N-terminal peptide membrane binding.

Le Brun AP, Haigh CL, Drew SC, James M, Boland MP, Collins SJ.

Biophys J. 2014 Nov 18;107(10):2313-24. doi: 10.1016/j.bpj.2014.09.027.


MEK1 transduces the prion protein N2 fragment antioxidant effects.

Haigh CL, McGlade AR, Collins SJ.

Cell Mol Life Sci. 2015 Apr;72(8):1613-29. doi: 10.1007/s00018-014-1777-y. Epub 2014 Nov 13.


Pratylenchus quasitereoides n. sp. from cereals in Western Australia.

Hodda M, Collins SJ, Vanstone VA, Hartley D, Wanjura W, Kehoe M.

Zootaxa. 2014 Sep 23;3866(2):277-88. doi: 10.11646/zootaxa.3866.2.6.


Ascertainment bias causes false signal of anticipation in genetic prion disease.

Minikel EV, Zerr I, Collins SJ, Ponto C, Boyd A, Klug G, Karch A, Kenny J, Collinge J, Takada LT, Forner S, Fong JC, Mead S, Geschwind MD.

Am J Hum Genet. 2014 Oct 2;95(4):371-82. doi: 10.1016/j.ajhg.2014.09.003.


Effectiveness of the surgical safety checklist in correcting errors: a literature review applying Reason's Swiss cheese model.

Collins SJ, Newhouse R, Porter J, Talsma A.

AORN J. 2014 Jul;100(1):65-79.e5. doi: 10.1016/j.aorn.2013.07.024. Review.


Detection rates and phenotypic spectrum of m.3243A>G in the MT-TL1 gene: a molecular diagnostic laboratory perspective.

Chin J, Marotta R, Chiotis M, Allan EH, Collins SJ.

Mitochondrion. 2014 Jul;17:34-41. doi: 10.1016/j.mito.2014.05.005. Epub 2014 May 17.


C-terminal peptides modelling constitutive PrPC processing demonstrate ameliorated toxicity predisposition consequent to α-cleavage.

Johanssen VA, Johanssen T, Masters CL, Hill AF, Barnham KJ, Collins SJ.

Biochem J. 2014 Apr 1;459(1):103-15. doi: 10.1042/BJ20131378.


Antioxidant and Metal Chelation-Based Therapies in the Treatment of Prion Disease.

Brazier MW, Wedd AG, Collins SJ.

Antioxidants (Basel). 2014 Apr 21;3(2):288-308. doi: 10.3390/antiox3020288. Review.


Surveillance for Creutzfeldt-Jakob disease in Australia: update to December 2012.

Klug GM, Boyd A, Zhao T, Stehmann C, Simpson M, McLean CA, Masters CL, Collins SJ.

Commun Dis Intell Q Rep. 2013 Jun 30;37(2):E115-20.


Updated Creutzfeldt-Jakob disease infection control guidelines: sifting facts from fiction.

Collins SJ, Athan E, Koehler AP.

Med J Aust. 2013 Oct 21;199(8):535-6. No abstract available.


Intensity of human prion disease surveillance predicts observed disease incidence.

Klug GM, Wand H, Simpson M, Boyd A, Law M, Masters CL, Matěj R, Howley R, Farrell M, Breithaupt M, Zerr I, van Duijn C, Ibrahim-Verbaas C, Mackenzie J, Will RG, Brandel JP, Alperovitch A, Budka H, Kovacs GG, Jansen GH, Coulthard M, Collins SJ.

J Neurol Neurosurg Psychiatry. 2013 Dec;84(12):1372-7. doi: 10.1136/jnnp-2012-304820. Epub 2013 Aug 21.


Unusual clinical and molecular-pathological profile of gerstmann-Sträussler-Scheinker disease associated with a novel PRNP mutation (V176G).

Simpson M, Johanssen V, Boyd A, Klug G, Masters CL, Li QX, Pamphlett R, McLean C, Lewis V, Collins SJ.

JAMA Neurol. 2013 Sep 1;70(9):1180-5. doi: 10.1001/jamaneurol.2013.165.


Immunotherapeutic approaches in prion disease: progress, challenges and potential directions.

Brazier MW, Mot AI, White AR, Collins SJ.

Ther Deliv. 2013 May;4(5):615-28. doi: 10.4155/tde.13.30. Review. Erratum in: Ther Deliv. 2013 Jun;4(6):762.


Cytosolic caspases mediate mislocalised SOD2 depletion in an in vitro model of chronic prion infection.

Sinclair L, Lewis V, Collins SJ, Haigh CL.

Dis Model Mech. 2013 Jul;6(4):952-63. doi: 10.1242/dmm.010678. Epub 2013 Apr 4.


Updated Creutzfeldt-Jakob disease infection control guidelines: sifting facts from fiction.

Koehler AP, Athan E, Collins SJ.

Med J Aust. 2013 Mar 18;198(5):245-6. No abstract available.


Surveillance of Creutzfeldt-Jakob disease in Australia: update to December 2011.

Klug GM, Boyd A, McGlade A, Stehmann C, Simpson M, Masters CL, Collins SJ.

Commun Dis Intell Q Rep. 2012 Jun 30;36(2):E174-9.


The prion protein preference of sporadic Creutzfeldt-Jakob disease subtypes.

Klemm HM, Welton JM, Masters CL, Klug GM, Boyd A, Hill AF, Collins SJ, Lawson VA.

J Biol Chem. 2012 Oct 19;287(43):36465-72. doi: 10.1074/jbc.M112.368803. Epub 2012 Aug 28.


Preschoolers' sensitivity to speaker action constraints to infer referential intent.

Collins SJ, Graham SA, Chambers CG.

J Exp Child Psychol. 2012 Aug;112(4):389-402. doi: 10.1016/j.jecp.2012.03.008. Epub 2012 May 29.


Prion subcellular fractionation reveals infectivity spectrum, with a high titre-low PrPres level disparity.

Lewis V, Haigh CL, Masters CL, Hill AF, Lawson VA, Collins SJ.

Mol Neurodegener. 2012 Apr 26;7:18. doi: 10.1186/1750-1326-7-18.


Loss of maternal CTCF is associated with peri-implantation lethality of Ctcf null embryos.

Moore JM, Rabaia NA, Smith LE, Fagerlie S, Gurley K, Loukinov D, Disteche CM, Collins SJ, Kemp CJ, Lobanenkov VV, Filippova GN.

PLoS One. 2012;7(4):e34915. doi: 10.1371/journal.pone.0034915. Epub 2012 Apr 20.

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