Alzheimer's Disease: Advances in Etiology, Pathogenesis and Therapeutics

846 Pages·2001·9.42 MB·English

by  Iqubal K. (ed.)

Alzheimer’sDisease:AdvancesinEtiology,PathogenesisandTherapeutics
EditedbyKhalidIqbal,SangramS.SisodiaandBengtWinblad
Copyright&2001 JohnWiley&SonsLtd
PrintISBN0-471-52176-0 OnlineISBN0-470-84645-3
Alzheimer’s Disease
ADVANCES IN ETIOLOGY,
PATHOGENESIS AND THERAPEUTICS
Alzheimer’sDisease:AdvancesinEtiology,PathogenesisandTherapeutics
EditedbyKhalidIqbal,SangramS.SisodiaandBengtWinblad
Copyright&2001 JohnWiley&SonsLtd
PrintISBN0-471-52176-0 OnlineISBN0-470-84645-3
This publication was
supported by an educational grant from
Janssen Pharmaceutica and Janssen Research Foundation
Alzheimer’sDisease:AdvancesinEtiology,PathogenesisandTherapeutics
EditedbyKhalidIqbal,SangramS.SisodiaandBengtWinblad
Copyright&2001 JohnWiley&SonsLtd
PrintISBN0-471-52176-0 OnlineISBN0-470-84645-3
Alzheimer’s Disease
ADVANCES IN ETIOLOGY,
PATHOGENESIS AND THERAPEUTICS
Edited by
KHALID IQBAL
New York State Institute for Basic Research, New York, USA
SANGRAM S. SISODIA
University of Chicago, Chicago, USA
BENGT WINBLAD
Karolinska Institute, Stockholm, Sweden
JOHN WILEY & SONS, LTD
Chichester . New York . Weinheim . Brisbane . Singapore . Toronto
Alzheimer’sDisease:AdvancesinEtiology,PathogenesisandTherapeutics
EditedbyKhalidIqbal,SangramS.SisodiaandBengtWinblad
Copyright&2001 JohnWiley&SonsLtd
PrintISBN0-471-52176-0 OnlineISBN0-470-84645-3
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LibraryofCongressCataloging-in-PublicationData
Alzheimer’sdisease:advancesinetiology,pathogenesisand therapeutics/editedby
KhalidIqbal,SangramS.Sisodia,BengtWinblad
p. ; cm.
Includesbibliographicalreferencesandindex.
ISBN0-471-52176-0(cased:alk.paper)
1.Alzheimer’sdisease—Congresses. I.Iqbal,Khalid. II.Sisodia,SangramS.
III.Winblad,Bengt. IV.InternationalConferenceonAlzheimer’sDiseasesand
RelatedDisorders(7th:2000:Washington,D.C.)
[DNLM: 1.AlzheimerDisease—etiology—Congresses. 2.AlzheimerDisease—
pathology—Congresses. 3.AlzheimerDisease—therapy—Congresses.WT155A47572001]
RC523A37532001
616.8’31—dc21 00–067218
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ISBN0-471-52176-0
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Alzheimer’sDisease:AdvancesinEtiology,PathogenesisandTherapeutics
EditedbyKhalidIqbal,SangramS.SisodiaandBengtWinblad
Copyright&2001 JohnWiley&SonsLtd
PrintISBN0-471-52176-0 OnlineISBN0-470-84645-3
Contents
List of Contributors xii
Dedications xxvi
Scientists HonoredforPioneering Research xxix
Preface xxxv
Acknowledgments xxxvi
I. EPIDEMIOLOGYANDRISKFACTORS 1
1. The Transition from NormalFunctioning to Dementia
in theAgingPopulation
Laura Fratiglioni, BrentSmall, Bengt Winblad andLarsBa¨ckman 3
2. Epidemiology of Alzheimer’s Diseaseand Dementia:
Advances and Challenges
Robert Katzman 11
3. Epidemiology of DementiainDown’sSyndrome
Nicole Schupf 23
II. GENETICS 31
4. A GenomicSearch forAlzheimer’s Disease Genes
Jonathan L.Haines, L.Renee Bailey, Janet M.Grubber, DaleHedges,
Jenifer L. Hall,SandraWest, Leonard Santoro, BethKemmerer,
AnneM.Saunders, Allen D. Roses,Gary W.Small,William K.Scott,
P. Michael Conneally,Jeffery M.VanceandMargaret A. Pericak-Vance 33
5. Candidate Genes ShowingNo Evidenceof Association with
Alzheimer’s Disease: Results oftheNIMH-AD GeneticsInitiative
Lars Bertram, Deborah Blacker, AdamS. Crystal, Jennifer Jones,
Devon Keeney,Laura A. MacKenzie-Ingano, Kristina Mullin,Sanjay Basu,
Stephen Yhu, MelvinMcInnis, Rodney C.P.Go,Aleister J.Saunders
and RudolphE.Tanzi 45
6. Familial Alzheimer’s Diseasewith SpasticParaparesis Associated
with aMutation atCodon 261 ofthePresenilin 1Gene
Martin R.Farlow, Jill R.Murrell, Frederick W.Unverzagt, Michael
Phillips, Masaki Takao,Christine Hulette andBernardino Ghetti 53
7. Genetic Analysis ofthe PresenilinPathway inDrosophila
Izhar Livne-Bar andGabrielle L.Boulianne 61
8. Molecular Geneticsand TransgenicModeling oftheTauopathies
Jada Lewis,MattBaker, Marjon VanSlegtenhorst andMike Hutton 71
9. Regulation of Four-repeat tauExpression: Interactions
between ExonandIntron Splicing RegulatorySequences
Ian D’Souza andGerard D.Schellenberg 87
vi CONTENTS
III. DIAGNOSIS ANDCLINICAL COURSE 97
10. Preclinical Prediction ofAD:Relation BetweenNeuropsychological
and Neuroimaging Findings
Marilyn S. Albert, Ronald J.Killiany,Keith Johnson, RudolphE.Tanzi
andKenneth Jones 99
11. Neuropsychological Detectionof Preclinical Alzheimer’s Disease:
Results ofa Neuropathological Seriesof‘Normal’ Controls
Kathleen A. Welsh-Bohmer, Christine Hulette, Donald Schmechel,
James Burkeand AnnSaunders 111
12. Potentially Reversible Conditions inMemory ClinicPatients
Anne-Mette Hejl, PeterHøgh andGunhild Waldemar 123
13. The Alzheimer’s Disease Centers’ Neuropsychological Database
Initiative: AResource forAlzheimer’s DiseasePrevention Trials
Michael Grundman, Hyun T.Kim, David Salmon,Martha Storandt,
Glenn Smith, StevenFerris, Richard Mohs, JasonBrandt, Rachelle
Doody,Kathleen Welsh-Bohmer, Judith Saxton,Kathy Saine,
Frederick Schmitt, Paula Ogrocki, NancyJohnson, DianeHowieson,
Michelle Papka,Joanne Green, Anthony Gamst,Walter Kukull and
Leon J.Thal, fortheAlzheimer’s Disease Centers’ Neuropsychological
Database Initiative 129
14. Mild Cognitive Impairment: Transition from Agingto Alzheimer’s
Disease
Ronald C. Petersen 141
15. Brain FunctionalImaging inEarlyand Preclinical Alzheimer’s
Disease
Agneta Nordberg,Vesna Jelic,EvaArna´iz, Bengt La˚ngstro¨m andOveAlmkvist 153
16. Amyloid, PHF-tau, Ubiquitinand Synaptic Markersinthe
Progression ofAlzheimer’s Disease: Immunochemical Analyses
of FrontalCortex from ProspectivelyStudied Elderly Humans
D. S. Wang,E.Cochran, D.Bennett, E.Mufson, C.Eckman
andD. W.Dickson 165
17. Imaging theConsequences of Alzheimer’s DiseasePathology
Yaakov Stern andScottSmall 181
18. Influence ofapoE Genotype andPET BrainImaging on
Preclinical Prediction ofAlzheimer’s Disease
Gary W.Small,Linda M.Ercoli, DanielH.S.Silverman, S.-C.Huang,
Susan Y.Bookheimer, Helen Lavretsky, KarenMiller, Prabha Siddarth,
John C.Mazziotta, AnnM.Saunders, MargaretA. Pericak-Vance,
Allen D. Roses,Jorge R.Barrio andMichael E.Phelps 193
19. Overview ofVascular Dementia
William R. Markesbery 205
20. Clinical and Imaging Characteristics ofVascular Dementiain
a Memory Clinic
F. Pasquier, X. Douay,C. Delmaire,F. LebertandJ.P. Pruvo 219
21. MRI ofEntorhinal Cortex andHippocampus inAlzheimer’s
Disease, Subcortical Ischemic Vascular Dementiaand
Mixed Dementia
N. Schuff, A.T. Du,D. Amend,Y. Y. Hsu, M.P.Laakso, W.Jagust,
H. C.Chui andM.W.Weiner 229
CONTENTS vii
22. Olfactory Function and Event-related Potentials inAlzheimer’s
Disease
Claire Murphy andCharlieD. Morgan 237
23. Phenotypic Differences in CholinergicMarkerswithinthe
Nucleus BasalisinIndividuals withMild Cognitive Impairment
Elliott J.Mufson, Michele Gilmor, Shuang Y.Ma, AlanI.Levey
and JeffreyH.Kordower 253
24. Evaluating CNSBiomarkers forAlzheimer’s Disease
John H.Growdon 265
25. CSFMarkersfor EarlyAlzheimer’s Disease
Kaj Blennow, Pia Davidsson andEugeen Vanmechelen 275
26. CSF-Phospho-tau (181P) asa PromisingMarkerfor Discriminating
Alzheimer’s Disease from Dementiawith LewyBodies
E.Vanmechelen, E.Van Kerschaver, K.Blennow, P.P. DeDeyn,D. Galasko,
L. Parnetti, C.J.M.Sindic,H.Arai, M.Riemenschneider, H. Hampel,
H. Pottel, A.Valgaeren, F.Hulstaert andH.Vanderstichele 285
27. Increased Levelsof aMinor Glycoform ofAcetylcholinesterase
in Alzheimer’s DiseaseBrain andCerebrospinal Fluid
Javier Sa´ez-Valero, SuSan Mok,LisaFodero, Alberto Marcos,
Maria-Sagrario Barquero, Catriona McLean andDavidH. Small 293
28. Plasma b-Amyloid asa SurrogateGenetic Marker inLate-onset
Alzheimer’s Disease
Nilufer Ertekin Taner, Neill Graff-Radford, Linda H. Younkin,
Christopher Eckman, Jennifer Adamson,DanielJ.Schaid, John Blangero,
Michael Hutton andSteven G.Younkin 303
29. Levels ofTotal and Deposited Abare Correlated with Dementia
S. Parvathy, J.Naslund, V.Haroutunian andJ.D. Buxbaum 311
30. WhatShould WeTell PatientsAttendinga Memory Disorders
Clinic AboutTheir Diagnosis?
Conor P.Maguire andRebecca M.Slinn 319
IV. MECHANISMSOF NEURODEGENERATION 329
31. Innate Immunity,Autotoxicity andDegenerative Neurologies
Patrick L.McGeer, KojuYasojima andEdith G. McGeer 331
32. Neuroinflammatory Responses intheAlzheimer’s DiseaseBrain
Promote theOxidativePost-translational Modificationof
Amyloid Deposits
Craig S. Atwood, Xudong Huang,Robert D. Moir, MarkA. Smith,
Rudolph E.Tanzi, AlexE.Roher, Ashley I.Bushand GeorgePerry 341
33. Plasma Antioxidantsand OxidativeDNA Damage inLymphocytes
from Normal AgedPeople andAlzheimer’s Disease Patients
Patrizia Mecocci,MariaCristina Polidori, Tiziana Ingegni, PaolaMattioli,
Antonio Cherubini, Marco Catani,Roberta Cecchetti andUmberto Senin 363
34. Oxidative Damageand Antioxidant Responses inAlzheimer’s
Disease
George Perry, AkihikoNunomura, Jesus Avila, MarPerez,
Catherine A.Rottkamp, Craig S. Atwood, Xiongwei Zhu,Gjumrakch Aliev,
Adam D.Cashand MarkA. Smith 371
viii CONTENTS
35. Cyclooxygenase (COX)-2 andClinical Progression ofAlzheimer’s
Disease Dementia:Implications intheRole ofNeuronal COX-2
in CellCycle
Giulio MariaPasinetti 379
36. Parallels betweenthe Redox Propertiesand ToxicityofAb in
Alzheimer’s Disease andMutant Cu/Zn-SODin Familial
Amyotrophic LateralSclerosis
Ashley I.Bush 393
37. b-Amyloid Toxicity:Diverse Biological Activities DriveMultiple
Cellular Mechanisms
C.W.Cotman, L.Tong, A. Anderson,D. Cribbsand J.Su 407
38. Mechanisms ofAb Production and AbDegradation: Routesto
theTreatment ofAlzheimer’s Disease
D. J.Selkoe,W.Xia, W.T. Kimberly, K.Vekrellis,D. Walsh,
W.P. EslerandM.S. Wolfe 421
39. A HighFat,HighCholesterol DietAccelerates b-Amyloid
Accumulation in theCNSof aTransgenic MouseModel of
Alzheimer’s Disease
Lorenzo M.Refolo, BrianMalester, JohnLaFrancois, Tara Bryant-Thomas,
Rong Wang,G.Stephen Tint, Kumar Sambamurti, KarenDuffand
Miguel A. Pappolla 433
40. Electron Microscopy and X-rayDiffractionStudies furtherConfirm
theEfficacy ofPTI-00703TM1 (Cat’sClaw Derivative)as a
Potential InhibitorofAlzheimer’s b-Amyloid Protein Fibrillogenesis
Gerardo M.Castillo, DanielA.Kirschner, AnnG.Yee andAlanD. Snow 449
41. Accelerated Ab Generation ina Cell Model ofAlzheimer’s
Disease-related Endosomal–Lysosomal SystemUpregulation
PaulM.Mathews, Carolyn B. Guerra, Ying Jiang, Benjamin H.Kao,
Ravi Dinakar, Pankaj Mehta,AnneM.Cataldo andRalph A.Nixon 461
42. The Amyloid Precursor ProteinV717I MutationIncreases
Susceptibility to Cell Deathina Cholesterol-dependent Manner
Luigi Puglielli, Laura A.MacKenzie Ingano, RudolphE.Tanzi and
Dora M.Kovacs 469
43. Intracellular andSecreted Ab Ratios AreDifferently
42/40
Influenced byAPPMutations
Heike S. Grimm, StefanF. Lichtenthaler, Konrad Beyreuther
andTobias Hartmann 479
44. Familial BritishDementia
Jorge Ghiso, TamasRe´ve´sz,AguedaRostagno, RubenVidal,
Gordon Plantand Blas Frangione 487
45. Cellular Metabolism ofFamilialBritishDementia-associated
BRI-L
Seong-Hun KimandSangram S. Sisodia 495
46. A DecamerDuplication inthe BRIGene Originatesa denovo
Amyloid Peptide thatCauses Dementiaina Danish Kindred
RubenG. Vidal, TamasRe´ve´sz,AguedaRostagno, Toke Bek,
Hans Braendgaard, Gordon Plant, JorgeGhiso andBlas Frangione 507
47. Familial Alzheimer’s Disease-linkedMutant
Presenilins Attenuate Capacitative CalciumEntry
Isaac Cheng,AndrewS. Yoo,Rudolph E.TanziandTae-Wan Kim 515
CONTENTS ix
48. Presenilin-1 Is a Regulatory Component of the Cadherin Cell Adhesion
Complex: Implications forAlzheimer’s Disease
Anastasio Georgakopoulos, PhilippeMarambaud, Nikolaos K.Robakis
and Lia Baki 521
49. Presenilins and Notch SignalingPathway
Weihong Song andBruceA.Yankner 531
50. Functional Consequences oftheAssociation of PS1withb-Catenin
Salvador Soriano, David E.Kang, Nathalie Chevallier, HuiZheng
and Edward H.Koo 541
51. A Novel ProteaseActive Site MotifConserved inPresenilins
and Polytopic Bacterial Aspartyl Proteases?
Harald Steiner andChristian Haass 549
52. The Unfolded Protein Response-mediated Upregulation ofBiP
and CHOP IsnotAffected byPresenilin Expression
Naoyuki SatoandGopal Thinakaran 559
53. Mechanisms ofa-Synuclein and NACFibrillogenesis
Makoto Hashimoto, Edward Rockenstein, Takato Takenouchi,
Margaret Mallory andEliezer Masliah 569
54. Neurofibrillary Degeneration: PatternsofTau IsoformExpression
Andre´Delacourte 587
55. Phosphorylation, Microtubule Bindingand Aggregation ofTau
Protein inAlzheimer’s Disease
Jesu´sA´vila,Jose´J.Lucas,Filip Lim, Mar Pe´rez, Fe´lix Herna´ndez,
Montserrat Arrasate,Rosario Armas Portela,Elsa Champion, GeorgePerry,
Mark A.Smith andJavierDı´azNido 601
56. Phosphorylation ofProtein Tauand Rescue ofProtein
Tau-inducedAxonopathybyGSK-3binGSK-3b6htau40
DoubleTransgenicMice
KurtSpittaels,ChrisVandenHaute,JoVanDorpe,HugoGeerts
andFredVanLeuven 609
57. Pathogenic Implicationof AlteredTau PropertiesCaused by
FTDP-17 Mutations
P. Nacharaju, S. Yen,M.DeTure, C.Easson,M.Hutton
and S.-H.Yen 621
58. A Hexapeptide Motif(306VQIVYK311)-forming b Structure Induces
the Aggregationof TauProtein toPaired HelicalFilaments
M.von Bergen, J.Biernat, E.-M. MandelkowandEckhard Mandelkow 631
V. ANIMAL AND CELLULARMODELS 641
59. Formation of NeurofibrillaryTangles in MouseBrain
Akihiko Takashima andKentaro Tanemura 643
60. Inducible TransgenicExpression ofWild-type tau inH4
Neuroglioma Cells
Michael DeTure, Li-Wen Ko,Colin Easson,Mike Hutton andShu-Hui Yen 651
61. Lewy-like Pathology inMice TransgenicforMutant (A53T)
and Wild-type Human a-Synuclein
Bernd Sommer,Samuel Barbieri, KatjaHofele, Karl-Heinz Wiederhold,
Alphonse Probst,Claudia Mistl, SimoneDanner, SabineKauffmann,
Willibrordus Spooren, MarkusTolnay, GraemeBilbe and
Herman vanderPutten 661
x CONTENTS
62. Somal andNeuritic Accumulation of theParkinson’s
Disease-associated Mutant [A30P]a-Synuclein in TransgenicMice
Phillipp J.Kahle,Manuela Neumann, LaurenceOzmen,
Hans A. Kretzschmar andChristian Haass 671
63. Ex vivo Transmission ofMouse-adapted Prion Strains toN2a
and GT1-7Cell Lines
Sylvain Lehmann,Hubert Laude,David A. Harris, Richard I.Carp,
Didier Vilette,Shigeru Katamine, Jean-Yves Madec andNoriyuki Nishida 679
64. InVivo Perturbation ofLysosomalFunction Promotes
Neurodegeneration inthe PS1 /APP MouseModel
M146V K670N,M671L
of Alzheimer’s DiseasePathology
Ralph A. Nixon,Paul M.Mathews, AnneM.Cataldo,Panaiyur S. Mohan,
Stephen D. Schmidt, KarenDuff, Martin Berg,Neville Marks,
Corinne Peterhoff andHenry Sershen 687
65. Changes inCognitive Characteristics ofTg(APP)CRND8Mice
atEarlyStages ofImmunization withBeta-Amyloid Peptide
Christopher Janus, Jacqueline Pearson, Patrick Horne,Richard Renlund,
Karen Parisien, AzharChishti, DonnaHeslin, CatherineBergeron, PaulFraser,
Peter StGeorge-Hyslop andDavid Westaway 697
VI. THERAPEUTICS AND THERAPEUTICSTRATEGIES 705
66. Galantamine, aNovel Treatment forAlzheimer’s Disease:
A ReviewofLong-term Benefits toPatients and Caregivers
Pierre TariotandBengt Winblad 707
67. Benefits ofDonepezil on Cognition,Function and
Neuropsychiatric Symptomsin Patientswith Mild andModerate
Alzheimer’s Disease over OneYear
Gunhild Waldemar, Bengt Winblad, Knut Engedal, Hilkke Soininen,
Frans Verhey, AndersWimo, Anne-Lena Wetterholm, Richard Zhang,
AndersHaglund, PonniSubbiah andthe DonepezilNordic StudyGroup 725
68. Characterization ofAlzheimer’s b-Secretase ProteinBACE:
Processing and OtherPost-translational Modifications
Mitsuru Haniu,Brian D. Bennett,PaulDenis,Yunjen Young,
Elizabeth A.Mendiaz, JanisFuller, JohnO. Hui, StevenKahn,
Safura Babu-Khan, Sandra Ross,Teresa Burgess, Viswanatham Katta,
Margery Nicolson, Jonathan Lull, Shue-Yuan Wang,GaryRogers,
Robert Vassar andMartin Citron 739
69. AndrogenTreatment Reduces Cognitive DeficitsinFemale
apoE4 TransgenicMice
Jacob Raber, Anthony LeFevour andLennart Mucke
70. Studies withtheMemory-enhancing DrugAIT-082 in PC12Cells
Debomoy K.Lahiri, Yuan-Wen Ge andMartin R. Farlow 747
71. Generation ofAuto-antibodies toward Alzheimer’s Disease
Vaccination
Beka Solomonand DanFrenkel 759
72. Toward theIdentification ofc-Secretase: Using Transition State
Analog Inhibitors
William P. Esler, W.Taylor Kimberly, BethL.Ostaszewski,
Weiming Xia, Dennis J.Selkoe andMichael S.Wolfe 777