Prions : Methods and Protocols

368 Pages·2017·7.532 MB·English

by  Lawson, Victoria A

Methods in
Molecular Biology 1658
Victoria A. Lawson Editor
Prions
Methods and Protocols
M M B
ETHODS IN OLECULAR IO LO GY
SeriesEditor
JohnM.Walker
School of Lifeand MedicalSciences
University ofHertfordshire
Hatfield, Hertfordshire,AL109AB,UK
Forfurther volumes:
http://www.springer.com/series/7651
Prions
Methods and Protocols
Edited by
Victoria A. Lawson
Department of Pathology
The University of Melbourne
Parkville, VIC, Australia
Editor
VictoriaA.Lawson
DepartmentofPathology
TheUniversityofMelbourne
Parkville,VIC,Australia
ISSN1064-3745 ISSN1940-6029 (electronic)
MethodsinMolecularBiology
ISBN978-1-4939-7242-5 ISBN978-1-4939-7244-9 (eBook)
DOI10.1007/978-1-4939-7244-9
LibraryofCongressControlNumber:2017952947
©SpringerScience+BusinessMediaLLC2017
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Preface
The transmissible spongiform encephalopathy or prion diseases are a group of invariably
fatalneurodegenerativedisorderswithsporadic,familialandacquiredetiologiesthataffecta
varietyofanimalspeciesincludinghumans.
Itisnowgenerallyacceptedthatpriondiseasesarisethroughtheaberrantmisfoldingof
thehost-encodedcellularprionprotein.Proteinmisfolding,althoughnotuniqueamongst
neurodegenerative disorders, can in the case of prion diseases be seeded by the misfolded
protein,apropertywhichimpartsthetransmissiblenatureofpriondiseasesintheabsenceof
aconventionalinfectiousagent.Itisthisfeature,inconjunctionwithdifficultiesassociated
withdecontaminationofinfectedsurgicalinstrumentsandmaterial,whichcanbeattributed
to the level of community interest and public health concerns despite the diseases’ low
prevalence.
It has been over 30 years since a novel infectious particle was proposed to be the
causativeagentoftheprototypicpriondisease,scrapie.Provingtheexistenceofatransmis-
sibleproteinor‘prion’andunderstandingpriondiseasetransmissionandpathogenesishave
led to the development of many innovative methodologies. Understanding the unique
natureofthediseasehasledtoatrulymultidisciplinaryapproach,whichunitesbiochemical
and biophysical approaches with in vitro and in vivo models and animal biologists and
veterinarianswithbiomedicalresearchersandclinicians.
This book brings together protocols from each of these disciplines and highlights the
contributioneachdisciplinehasmadetoour understandingofthenatureofpriondisease.
In addition to contributing to our understanding of prion disease, these methods may
alsofindapplicationtothenewlyemergingandso-called‘prion-like’propertiesobservedin
other protein misfolding neurodegenerative diseases as highlighted in the final method
whichdescribesamethodfordetectingtheintercellularprion-likeconversionandtransmis-
sionofCu/Znsuperoxidedismutase(SOD1).
Parkville,VIC,Australia VictoriaA.Lawson
v
Contents
Preface ..................................................................... v
Contributors................................................................. ix
PART I METHODS FOR BIOCHEMICAL AND BIOPHYSICAL ANALYSIS
OF PRION DISEASE
1 PurificationandFibrillationofFull-LengthRecombinantPrP................ 3
NatalliaMakarava,ReginaSavtchenko,andIliaV.Baskakov
2 MethodforFoldingofRecombinantPrionProteintoSoluble
β-SheetSecondaryStructure ............................................. 23
LauraJ.Ellett
3 AnalysisofPrionProteinConformationUsingCircularDichroism
Spectroscopy ........................................................... 27
LauraJ.EllettandVanessaA.Johanssen
4 AnalysisofPrionProteinStructureUsingNuclearMagnetic
ResonanceSpectroscopy................................................. 35
IvanaBiljan,GregorIlc,andJanezPlavec
5 ImmunodetectionofPrPScUsingWesternImmunoblottingTechniques....... 51
GeraldS.BaronandGregoryJ.Raymond
6 AnalysisofmiRNASignaturesinNeurodegenerativePrionDisease ........... 67
ShayneA.BellinghamandAndrewF.Hill
PART II METHODS FOR CELLULAR ANALYSIS OF PRION DISEASE
7 CellBiologyApproachestoStudyingPrionDiseases ........................ 83
SuzetteA.Priola
8 ExpressionofHeterologousPrPandPrionPropagationinRK13Cells........ 95
ZairaE.Arellano-Anaya,AlvinaHuor,PascalLeblanc,
OlivierAndre´oletti,andDidierVilette
9 GenerationofInfectiousPrionsandDetectionwiththePrion-Infected
CellAssay.............................................................. 105
LauraJ.Vella,BradleyColeman,andAndrewF.Hill
10 AnalysisofCellularPrionProteinEndoproteolyticProcessing................ 119
VictoriaLewis
11 CellularAnalysisofAdultNeuralStemCellsforInvestigatingPrionBiology... 133
CathrynL.Haigh
12 NeurotoxicityofPrionPeptidesonCulturedCerebellarNeurons............. 147
GiuseppeD.Ciccotosto,MettaJana,andRobertoCappai
vii
viii Contents
PART III METHODS FOR CELL FREE PROPAGATION OF PRIONS
13 MethodsofProteinMisfoldingCyclicAmplification ........................ 169
NatalliaMakarava,ReginaSavtchenko,andIliaV.Baskakov
14 RT-QuICAssaysforPrionDiseaseDetectionandDiagnostics ............... 185
ChristinaD.Orru`,BradleyR.Groveman,AndrewG.Hughson,
MatteoManca,LynneD.Raymond,GregoryJ.Raymond,
KatrinaJ.Campbell,KelsieJ.Anson,AllisonKraus,andByronCaughey
15 AQuickMethodtoEvaluatetheEffectoftheAminoAcid
SequenceintheMisfoldingPronenessofthePrionProtein.................. 205
NataliaFerna´ndez-Borges,HasierEran˜a,SaioaR.Elezgarai,
ChafikHarrathi,VanesaVenegas,andJoaquı´nCastilla
PART IV IN VIVO INVESTIGATION OF PRION DISEASE
16 InsightsintoMechanismsofTransmissionandPathogenesis
fromTransgenicMouseModelsofPrionDiseases .......................... 219
JulieA.MorenoandGlennC.Telling
17 InVivo-NearInfraredImagingofNeurodegeneration ...................... 253
VictoriaA.Lawson,CarolinTumpach,CathrynL.Haigh,
andSimonC.Drew
18 StrainTypingofPrionDiseasesUsingInVivoMouseModels ............... 263
AileenBoyle,KrisHogan,JeanC.Manson,andAbigailB.Diack
19 PreparationandImmunostainingoftheMyentericPlexus
ofPrion-InfectedMice .................................................. 285
LauraJ.EllettandVictoriaA.Lawson
PART V METHODS FOR THE DETECTION AND TREATMENT OF PRION DISEASES
20 CellCultureMethodsforScreeningofPrionTherapeutics................... 295
HilaryE.McMahon
21 Real-TimeQuaking-InducedConversionforDiagnosisofPrionDisease ...... 305
KatsuyaSatoh,RyuichiroAtarashi,andNoriyukiNishida
22 MethodsforMolecularDiagnosisofHumanPrionDisease.................. 311
JonathanD.F.Wadsworth,GaryAdamson,SusanJoiner,
LaraBrock,CarolinePowell,JacquelineM.Linehan,
JonathanA.Beck,SebastianBrandner,SimonMead,andJohnCollinge
23 MolecularSubtypingofPrPresinHumanSporadicCJDBrainTissue ......... 347
G.M.Klug,V.Lewis,andS.J.Collins
PART VI METHODS FOR INVESTIGATION OF PRION-LIKE DISEASE
24 IntercellularPrion-LikeConversionandTransmissionofCu/Zn
SuperoxideDismutase(SOD1)inCellCulture............................. 357
LeslieI.Grad,EdwardPokrishevsky,andNeilR.Cashman
Index ...................................................................... 369
Contributors
GARYADAMSON (cid:1) MRCPrionUnitatUCL,UCLInstituteofPrionDiseases,London,UK
OLIVIER ANDRE´OLETTI (cid:1) INRA,UMR1225,IHAP,Toulouse,France;Universite´ de
Toulouse,INP,ENVT,UMR1225,IHAP,Toulouse,France
KELSIEJ.ANSON (cid:1) LaboratoryofPersistentViralDiseases,RockyMountainLaboratories,
NIAID,NIH,Hamilton,MT,USA
ZAIRAE.ARELLANO-ANAYA (cid:1) INRA,UMR1225,IHAP,Toulouse,France;Universite´ de
Toulouse,INP,ENVT,UMR1225,IHAP,Toulouse,France
RYUICHIROATARASHI (cid:1) DivisionofMicrobiology,DepartmentofInfectiousDiseases,Facultyof
Medicine,UniversityofMiyazaki,Miyazaki,Japan
GERALD S.BARON (cid:1) RockyMountainLaboratories,NationalInstituteofAllergyand
InfectiousDiseases,NIH,Hamilton,MT,USA
ILIAV.BASKAKOV (cid:1) DepartmentofAnatomyandNeurobiology,Center forBiomedical
EngineeringandTechnology,UniversityofMarylandSchoolofMedicine,Baltimore,MD,
USA
JONATHANA.BECK (cid:1) MRCPrionUnitatUCL,UCLInstituteofPrionDiseases,
London,UK
SHAYNE A.BELLINGHAM (cid:1) DepartmentofBiochemistryandMolecularBiology,Bio21
MolecularScienceandBiotechnologyInstitute,TheUniversityofMelbourne,Parkville,
VIC,Australia
IVANABILJAN (cid:1) DepartmentofChemistry,FacultyofScience,UniversityofZagreb,Zagreb,
Croatia
AILEENBOYLE (cid:1) TheRoslinInstitute&R(D)SVS,UniversityofEdinburgh,Edinburgh,UK
SEBASTIANBRANDNER (cid:1) MRCPrionUnitatUCL,UCLInstituteofPrionDiseases,
London,UK
LARA BROCK (cid:1) MRCPrionUnitatUCL,UCLInstituteofPrionDiseases,London,UK
KATRINAJ.CAMPBELL (cid:1) LaboratoryofPersistentViralDiseases,RockyMountain
Laboratories,NIAID,NIH,Hamilton,MT,USA
ROBERTOCAPPAI (cid:1) DepartmentofPathology,TheUniversityofMelbourne,Parkville,VIC,
Australia
NEILR.CASHMAN (cid:1) DivisionofNeurology,DepartmentofMedicine,UBCHospital,
UniversityofBritishColumbia,Vancouver,BC,Canada
JOAQUI´NCASTILLA (cid:1) CICbioGUNE,ParqueTecnolo´gicodeBizkaia,Derio,Bizkaia,Spain;
IKERBASQUE,BasqueFoundationforScience,Bilbao,Bizkaia,Spain
BYRONCAUGHEY (cid:1) LaboratoryofPersistentViralDiseases,RockyMountainLaboratories,
NIAID,NIH,Hamilton,MT,USA
GIUSEPPE D.CICCOTOSTO (cid:1) DepartmentofPathology,TheUniversityofMelbourne,
Parkville,VIC,Australia
BRADLEYCOLEMAN (cid:1) DepartmentofBiochemistryandMolecularBiology,Bio21Molecular
ScienceandBiotechnologyInstitute,UniversityofMelbourne,Parkville,VIC,Australia
JOHNCOLLINGE (cid:1) MRCPrionUnitatUCL,UCLInstituteofPrionDiseases,London,UK
S.J.COLLINS (cid:1) DepartmentofMedicine,RoyalMelbourneHospital,TheUniversityof
Melbourne,Parkville,VIC,Australia
ix
x Contributors
ABIGAILB.DIACK (cid:1) TheRoslinInstitute&R(D)SVS,UniversityofEdinburgh,
Edinburgh,UK
SIMONC.DREW (cid:1) DepartmentofMedicine(RoyalMelbourneHospital),TheUniversityof
Melbourne,Parkville,VIC,Australia
SAIOAR.ELEZGARAI (cid:1) CICbioGUNE,ParqueTecnolo´gicodeBizkaia,Derio,Bizkaia,Spain
LAURA J.ELLETT (cid:1) DepartmentofPathology,TheUniversityofMelbourne,Parkville,VIC,
Australia
HASIERERAN˜A (cid:1) CICbioGUNE,ParqueTecnolo´gicodeBizkaia,Derio,Bizkaia,Spain
NATALIA FERNA´NDEZ-BORGES (cid:1) CICbioGUNE,ParqueTecnolo´gicodeBizkaia,Derio,
Bizkaia,Spain
LESLIEI.GRAD (cid:1) DivisionofNeurology,DepartmentofMedicine,UBCHospital,University
ofBritishColumbia,Vancouver,BC,Canada
BRADLEYR.GROVEMAN (cid:1) LaboratoryofPersistentViralDiseases,RockyMountain
Laboratories,NIAID,NIH,Hamilton,MT,USA
CATHRYNL.HAIGH (cid:1) DepartmentofMedicine,MelbourneBrainCentre,RoyalMelbourne
Hospital,TheUniversityofMelbourne,Parkville,VIC,Australia;LaboratoryofPersistent
ViralDiseases,RockyMountainLaboratories,NationalInstituteofAllergyandInfectious
Diseases,NationalInstitutesofHealth,Hamilton,MT,USA
CHAFIKHARRATHI (cid:1) CICbioGUNE,ParqueTecnolo´gicodeBizkaia,Derio,Bizkaia,Spain
ANDREWF.HILL (cid:1) DepartmentofBiochemistryandMolecularBiology,Bio21Molecular
ScienceandBiotechnologyInstitute,TheUniversityofMelbourne,Parkville,VIC,
Australia;DepartmentofBiochemistryandGenetics,LaTrobeInstituteforMolecular
Science,LaTrobeUniversity,Bundoora,VIC,Australia
KRISHOGAN (cid:1) TheRoslinInstitute&R(D)SVS,UniversityofEdinburgh,Edinburgh,UK
ANDREWG.HUGHSON (cid:1) LaboratoryofPersistentViralDiseases,RockyMountain
Laboratories,NIAID,NIH,Hamilton,MT,USA
ALVINAHUOR (cid:1) INRA,UMR1225,IHAP,Toulouse,France;Universite´ deToulouse,INP,
ENVT,UMR1225,IHAP,Toulouse,France
GREGORILC (cid:1) SlovenianNMRCentre,NationalInstituteofChemistry,Ljubljana,Slovenia;
EN-FISTCenterofExcellence,Ljubljana,Slovenia
METTAJANA (cid:1) DepartmentofPathology,TheUniversityofMelbourne,Parkville,VIC,
Australia
VANESSAA.JOHANSSEN (cid:1) DepartmentofMedicine,TheUniversityofMelbourne,Parkville,
VIC,Australia
SUSAN JOINER (cid:1) MRCPrionUnitatUCL,UCLInstituteofPrionDiseases,London,UK
G.M.KLUG (cid:1) DepartmentofMedicine,RoyalMelbourneHospital,TheUniversityof
Melbourne,Parkville,VIC,Australia
ALLISON KRAUS (cid:1) LaboratoryofPersistentViralDiseases,RockyMountainLaboratories,
NIAID,NIH,Hamilton,MT,USA
VICTORIA A.LAWSON (cid:1) DepartmentofPathology,TheUniversityofMelbourne,Parkville,
VIC,Australia
PASCALLEBLANC (cid:1) CNRS,UMR5239,LaboratoiredeBiologieMole´culairedelaCellule
(LBMC),EquipeDiffe´renciationNeuromusculaire,EcoleNormaleSupe´rieure-Lyon,Lyon
Cedex,France
VICTORIA LEWIS (cid:1) DepartmentofMedicine,RoyalMelbourneHospital,TheUniversityof
Melbourne,Parkville,VIC,Australia
JACQUELINEM.LINEHAN (cid:1) MRCPrionUnitatUCL,UCLInstituteofPrionDiseases,
London,UK
Contributors xi
NATALLIAMAKARAVA (cid:1) DepartmentofAnatomyandNeurobiology,Center forBiomedical
EngineeringandTechnology,UniversityofMarylandSchoolofMedicine,Baltimore,MD,
USA
MATTEOMANCA (cid:1) LaboratoryofPersistentViralDiseases,RockyMountainLaboratories,
NIAID,NIH,Hamilton,MT,USA
JEANC.MANSON (cid:1) TheRoslinInstitute&R(D)SVS,UniversityofEdinburgh,Edinburgh,UK
HILARY E.MCMAHON (cid:1) UCDSchoolofBiomolecularandBiomedicalScience,Conway
InstituteforBiomolecularandBiomedicalResearch,UniversityCollegeDublin,Dublin,
Ireland
SIMONMEAD (cid:1) MRCPrionUnitatUCL,UCLInstituteofPrionDiseases,London,UK
JULIEA.MORENO (cid:1) CellandMolecularBiologyGraduateProgram,Molecular,Cellularand
IntegrativeNeuroscienceGraduateProgram,DepartmentofMicrobiology,Immunology
andPathology,PrionResearchCenter,ColoradoStateUniversity,FortCollins,CO,USA
NORIYUKI NISHIDA (cid:1) DepartmentofMolecularMicrobiologyandImmunology,Nagasaki
UniversityGraduateSchoolofBiomedicalSciences,Nagasaki,Japan
CHRISTINAD.ORRU` (cid:1) LaboratoryofPersistentViralDiseases,RockyMountainLaboratories,
NIAID,NIH,Hamilton,MT,USA
JANEZPLAVEC (cid:1) SlovenianNMRCentre,NationalInstituteofChemistry,Ljubljana,
Slovenia;EN-FISTCenterofExcellence,Ljubljana,Slovenia;FacultyofChemistryand
ChemicalTechnology,UniversityofLjubljana,Ljubljana,Slovenia
EDWARDPOKRISHEVSKY (cid:1) DivisionofNeurology,DepartmentofMedicine,UBCHospital,
UniversityofBritishColumbia,Vancouver,BC,Canada
CAROLINEPOWELL (cid:1) MRCPrionUnitatUCL,UCLInstituteofPrionDiseases,
London,UK
SUZETTEA.PRIOLA (cid:1) LaboratoryofPersistentViralDiseases,RockyMountainLaboratories,
NationalInstituteofAllergyandInfectiousDiseases,NationalInstitutesofHealth,
Hamilton,MT,USA
GREGORYJ.RAYMOND (cid:1) RockyMountainLaboratories,NationalInstituteofAllergyand
InfectiousDiseases,NIH,Hamilton,MT,USA
LYNNED.RAYMOND (cid:1) LaboratoryofPersistentViralDiseases,RockyMountainLaboratories,
NIAID,NIH,Hamilton,MT,USA
KATSUYASATOH (cid:1) DepartmentofLocomotiveRehabilitationScience,NagasakiUniversity
GraduateSchoolofBiomedicalSciences,Nagasaki,Japan
REGINASAVTCHENKO (cid:1) DepartmentofAnatomyandNeurobiology,Center forBiomedical
EngineeringandTechnology,UniversityofMarylandSchoolofMedicine,Baltimore,MD,
USA
GLENNC.TELLING (cid:1) CellandMolecularBiologyGraduateProgram,Molecular,Cellular
andIntegrativeNeuroscienceGraduateProgram,DepartmentofMicrobiology,
ImmunologyandPathology,PrionResearchCenter,ColoradoStateUniversity,Fort
Collins,CO,USA
CAROLINTUMPACH (cid:1) FloreyInstituteofNeuroscienceandMentalHealth,Parkville,VIC,
Australia
LAURA J.VELLA (cid:1) TheFloreyInstituteofNeuroscienceandMentalHealth,Universityof
Melbourne,Parkville,VIC,Australia
VANESAVENEGAS (cid:1) CICbioGUNE,ParqueTecnolo´gicodeBizkaia,Derio,Bizkaia,Spain
DIDIERVILETTE (cid:1) INRA,UMR1225,IHAP,Toulouse,France;Universite´deToulouse,INP,
ENVT,UMR1225,IHAP,Toulouse,France
JONATHAND.F.WADSWORTH (cid:1) MRCPrionUnitatUCL,UCLInstituteofPrionDiseases,
London,UK