ABSTRACT

This authoritative volume contains 179 chapters by international experts on recent developments in our understanding of amyloid proteins, protein folding disorders, and new and proposed clinical trials in amyloidosis. Topics include detection and characterization techniques; biological functions; genetics; disorders, diagnosis, and treatments, incl

section Section 1|32 pages

Amyloid Fibrillogenesis

chapter |3 pages

In Silico to In Vitro Approach for Protein Folding and Misfolding

ByD. Gilis, Y. Dehouck, M. Rooman

chapter |3 pages

Status of Congo Red Stained Amyloid in Polarized Light

ByBart Kahr, Werner Kaminksy, Kacey Claborn, Miki Kurimoto, Lee-Way Jin

chapter |3 pages

Analysis of the Interactions Between Amyloid Fibrils and Lipid Components

ByG. P. Gellermann, E. Niekrasz, K. Gührs, S. Shtrasburg, M. Pras, R. P. Linke, M. Fändrich, S. Diekmann, T. R. Appel

chapter |3 pages

Disaggregation Experiments as a Tool to Detect Protofibrillar Intermediates

ByMartino Calamai, Claudio Canale, Annalisa Relini, Massimo Stefani, Fabrizio Chiti, Christopher M. Dobson

chapter |3 pages

Correlation Between Stability and Propensity to form Amyloid-Like Fibrils

ByE. Žerovnik, S. Rabzelj, M. Kenig, V. Turk

chapter |3 pages

Tissue Detection and Characterization of Amyloid- is Proteomics the Answer?

ByMaria M. Picken, Vanitha Thulasiraman, Roger N. Picken, Lee Lomas

chapter |3 pages

The Conformation of Amyloid β-Peptide in the Presence of Fluorinated and Alkylated Nanoparticles

ByS. Rocha, A. F. Thünemann, M. C. Pereira, M. A. N. Coelho, H. Möhwald, G. Brezesinski

section Section 2|138 pages

AL Amyloidosis

chapter |2 pages

Systemic Equine AL Amyloidosis

ByCharles L. Murphy, Burnette Crombie, Teresa K. Williams, Sallie D. Macy, Deborah T. Weiss, Alan Solomon, Yvette S. Nout

chapter |3 pages

Radioimaging of Primary (AL) Amyloidosis with an Amyloid-Reactive Monoclonal Antibody

ByJ.S. Wall, M.J. Paulus, S.J. Kennel, S. Gleason, J. Baba, J. Gregor, D. Wolfenbarger, D.T. Weiss, A. Solomon

chapter |3 pages

Immunoglobulin Light Chain Variable Region Genes Demonstrate Evidence for Antigen Selection in Primary Amyloidosis

ByT. Prokaeva, J. E. Ward, E. Fingar, M. Kaut, P. Smith, C. O’Hara, L.H. Connors, M. Skinner, D.C. Seldin

chapter |3 pages

Detection and Characterization of Post-Translational Modifications in AL Light Chain Proteins by Mass Spectrometry

ByR. Théberge, A. Lim, Y. Jiang, LH Connors, J. Eberhard, M. Skinner, CE Costello

chapter |3 pages

Glycosylation Studies of Monoclonal Immunoglobulin Deposition Disorders

ByL. A. Omtvedt, L. Royle, G. Husby, K. Sletten, C. Radcliffe, D. J. Harvey, R. A. Dwek, P. M. Rudd

chapter |3 pages

Structural Localization of Light Chain Amyloidogenicity

ByJ.S. Wall, C.L. Murphy, A. Tomaszewski, B. Crombie, A. Solomon

chapter |3 pages

Aggressive Light Chain Amyloidosis Cases have Unstable Immunoglobulin Light Chains

ByL. A. Sikkink, E. M. Baden, S. R. Vance, K. J.-L. Riley, A.T. Moch, M. Ramirez-Alvarado

chapter |3 pages

Molecular Classification of Light Chain (AL) Amyloidosis by Gene Expression Profiling

ByR. S. Abraham, M. K. Manske, K. V. Ballman, A. Dispenzieri, T. L. Price-Troska, M. A. Gertz, R. Fonseca

chapter |3 pages

Analysis of B Cell Clonal Evolution in BM and PBL of AL and MM Patients Using Graphical Quantification of Lineage Trees

ByR. S. Abraham, M. K. Manske, A. Sohni, H. Edelman, N. Zuckerman, G. Shahaf, A. Dispenzieri, M. Q. Lacy, M. A. Gertz, R. Mehr

chapter |3 pages

In Vitro and In Vivo Mouse Models for AL Amyloidosis

ByJ.E. Ward, D.A. Brenner, J. Eberhard, R. Liao, L.H. Connors, C. O’Hara, D.C. Seldin

chapter |3 pages

Serum Cardiac Troponins and N-Terminal Pro-Brain Natriuretic Peptide: A Staging System for Primary Systemic Amyloidosis

ByA. Dispenzieri, R.A. Kyle, M.Q. Lacy, A.S. Jaffe, T.M. Therneau, S.R. Zeldenrust, M.A. Gertz

chapter |3 pages

Amyloid-Induced Myocardial Dysfunction Depends on Biochemical Characteristics of the Amyloidogenic Protein

ByGiovanni Palladini, Maria Joao Saraiva, Stefano Perlini, Laura Obici, Monia Vezzoli, Teresa Coelho, Hipólito Reis, Francesca Lavatelli, Giovanbattista Vadacca, Remigio Moratti, Giampaolo Merlini

chapter |3 pages

The Reduction of the Serum Concentration of the Amyloidogenic Light-Chain in Cardiac AL Results in Prompt Improvement of Myocardial Function and Prolonged Survival Despite Unalterd Amount of Myocardial Amyloid Deposits

ByGiovanni Palladini, Stefano Perlini, Monia Vezzoli, Vittorio Perfetti, Francesca Lavatelli, Ivana Ferrero, Laura Obici, Riccardo Caccialanza, Arthur R. Bradwell, Giampaolo Merlini

chapter |3 pages

Organ Survival in AL Amyloidosis with Renal Involvement

ByGiovanni Palladini, Vittorio Perfetti, Laura Obici, Riccardo Caccialanza, Francesca Lavatelli, Giampaolo Merlini

chapter |3 pages

Nutritional Status of Outpatients with AL Systemic Amyloidosis

ByRiccardo Caccialanza, Giovanni Palladini, Hellas Cena, Christina Vagia, Carla Roggi, Giampaolo Merlini

chapter |3 pages

Cardiac Troponins and N-Terminal Pro-Brain Natriuretic Peptide Predict Survival in Patients with Primary Systemic Amyloidosis Undergoing Peripheral Blood Stem Cell Transplant

ByA. Dispenzieri, R.A. Kyle, M.Q. Lacy, A.S. Jaffe, T.M. Therneau, M.F. Burrit, M.A. Gertz

chapter |2 pages

BNP as a Marker of Cardiac Involvement in Amyloidosis

ByM. Nordlinger, B. J. Magnani, M. Skinner, R. H. Falk

chapter |3 pages

Quantitative Serum Free Light-Chain Assay in the Diagnostic Evaluation of AL Amyloidosis

ByH. Akar, D.C. Seldin, B. Magnani, C. O’Hara, J.L. Berk, C. Schoonmaker, H. Cabral, L.M. Dember, V. Sanchorawala, L.H. Connors, R.H. Falk, M. Skinner

chapter |3 pages

AL Amyloidosis-Associated Hypothyroidism: Prevalence and Relationship to Nephrotic Syndrome

ByJ.S. Bhatia, V. Sanchorawala, D.C. Seldin, L.F. Casserly, J.L. Berk, R. Falk, M. Skinner, L.M. Dember

chapter |3 pages

Renal Involvement in Systemic Amyloidosis: Report of an Italian Retrospective Study

ByF. Bergesio, A.M. Ciciani, G. Palladini, R. Brugnano, D. Bizzarri, S. Pasquali, M. Salvadori

chapter |3 pages

Case Report: Unusual Clinical Presentation of Primary AL Amyloidosis

ByMaria Grazia Chiappini, Marco Di Girolamo, Giovanni Selvaggi, Konstantinos Giannakakis

chapter |2 pages

Serum Levels of Free Kappa and Lambda Light Chains in Patients with Systemic AL, AA, and ATTR Amyloidosis

ByJ. van Steijn, J. Bijzet, H. de Wit, B.P.C. Hazenberg, I.I. van Gameren, K. van de Belt, E. Vellenga

chapter |2 pages

Diagnostic Value of Free Kappa and Lambda Light Chains in Fat Tissue of Patients with Systemic AL Amyloidosis

ByB.P.C. Hazenberg, J. Bijzet, H. de Wit, J. van Steijn, E. Vellenga, M.H. van Rijswijk

chapter |3 pages

Experience with a Patient-Adapted Approach to Autologous Peripheral Blood Stem Cell Transplantation in AL Amyloidosis

ByV. Perfetti, G. Palladini, S. Siena, M. Bregni, M. Dinicola, V. Navazza, M. Magni, A.M. Gianni, G. Merlini

chapter |3 pages

Risk-Adapted Intravenous Melphalan with Adjuvant Thalidomide and Dexamethasone for Newly Diagnosed Untreated Patients with Systemic AL Amyloidosis: Interim Report of a Phase II Trial

ByRl Comenzo, P. Zhou, L. Reich, S. Costello, A. Quinn, S. Fircanis, L. Drake, C. Hedvat, J. Teruya-Feldstein, Da Filippa, M. Fleisher

chapter |3 pages

Thalidomide Plus Intermediate-Dose Dexamethasone in Refractory/Relapsed AL Patients

ByGiovanni Palladini, Vittorio Perfetii, Stefano Perlini, Laura Obici, Francesca Lavatelli, Rosangela Invernizzi, Franco Bergesio, Benedetto Comotti, Salvatore Iannaccone, Giampaolo Merlini

chapter |3 pages

VAD Chemotherapy Followed by Autologous Stem Cell Transplantation (ASCT) for the Treatment of Nephrotic Syndrome in Light Chain (AL) Amyloidosis

ByJ.B. Perz, S.O. Schonland, R.P. Linke, M. Zeier, M. Hundemer, A.D. Ho, H. Goldschmidt

chapter |3 pages

Superior Survival in Primary Systemic Amyloidosis Patients Undergoing Peripheral Blood Stem Cell Transplant: A Case Control Study

ByA. Dispenzieri, R.A. Kyle, M.Q. Lacy, T.M. Therneau, M.A. Gertz

chapter |3 pages

Treatment of AL Amyloidosis with Tandem Cycles of High Dose Melphalan and Autologous Stem Cell Transplantation

ByV. Sanchorawala, D. G. Wright, K. Quillen, R. Kunz, K.T. Finn, K. Malek, R. H. Falk, J. Berk, L.M. Dember, M. Skinner, D. C. Seldin

chapter |3 pages

The Use of Sequential Living Donor Kidney and Autologous Stem Cell Transplantation for Patients with Primary (AL) Amyloidosis and Advanced Renal Failure

ByN. Leung, M. D. Griffin, A. Dispenzieri, J. M. Gloor, T. R. Schwab, S. C. Textor, M. Q. Lacy, T. S. Larson, M. D. Stegall, M. A. Gertz

chapter |3 pages

Delayed Resolution of Nephrotic Syndrome Following Stem Cell Transplant for Amyloidosis (AL)

ByM. D. Morie Gertz, M. D. Nelson Leung, M. D. Martha Lacy, M. D. Angela Dispenzieri

chapter |3 pages

Amyloid Versus Non-Amyloid Immunoglobulin Deposits

ByG. Merlini

chapter |3 pages

Internalization of Systemic Amyloid Light Chains and Heparan Sulfate Proteoglycan Deposition

ByV. Trinkaus-Randall, S. Steeves, G. Monis, M.T. Walsh, J.D. Chiu, J. Eberhard, L.H. Connors, M. Skinner

chapter |3 pages

Human Amyloidogenic Light Chains Directly Invoke Oxidant Stress and Result in Impaired Cardiomyocyte Function

ByDaniel A. Brenner, Mohit Jain, David R. Pimentel, Wang Bo, Lawreen H. Connors, Martha Skinner, Carl S. Apstein, Ronglih Liao

chapter |3 pages

Management of Primary Amyloidosis (AL)

ByM. D. Morie Gertz

chapter |3 pages

Definition of Organ Involvement and Treatment Response in Primary Systemic Amyloidosis (AL): A Consensus Opinion from the 10TH International Symposium on Amyloid and Amyloidosis

ByMorie A. Gertz, Ray Comenzo, Rodney H. Falk, Jean Paul Fermand, Bouke P. Hazenberg, Philip N. Hawkins, Giampaolo Merlini, Philippe Moreau, Pierre Ronco, Vaishali Sanchorawala, Orhan Sezer, Alan Solomon, Giles Grateau

chapter |3 pages

Measurement of Serum Free Light Chains in AL Amyloidosis

ByH.D. Carr-Smith, R. Abraham, G.P. Mead, H. Goodman, P. Hawkins, A.R. Bradwell

chapter |3 pages

Melphalan Plus High-Dose Dexamethasone in AL Patients Who are Not Candidates for Stem Cell Transplantation

ByGiovanni Palladini, Vittorio Perfetii, Laura Obici, Francesca Lavatelli, Riccardo Caccialanza, Fausto Adami, Giobatta Cavallero, Roberto Rustichelli, Giovambattista Virga, Giampaolo Merlini

chapter |3 pages

High Dose Melphalan and Autologous Stem Cell Transplantation (HDM/SCT) for Treatment of AL Amyloidosis: Ten Year Single Institutional Experience

ByD. C. Seldin, V. Sanchorawala, K. Malek, D. G. Wright, K. Quillen, K. T. Finn, R. Falk, J. Berk, L. Dember, J. Wiesman, J. J. Anderson, M. Skinner

chapter |1 pages

Thalidomide Treatment in 80 Patients with AL Amyloidosis: Tolerability, Clonal Disease Response and Clinical Outcome

ByH.J.B. Goodman, H.J. Lachmann, R. Gallimore, P.N. Hawkins

section Section 3|124 pages

AA Amyloidosis

chapter |3 pages

Characteristic and Clinical Outcome of 340 Patients with Systemic AA Amyloidosis

ByH.J. Lachmann, H.J.B. Goodman, J. Gallimore, J.A. Gilbertson, J. Joshi, M.B. Pepys, P.N. Hawkins

chapter |3 pages

SAA1 Gene Analysis in Finnish Patients with AA Amyloidosis

ByC. Terai, H. Kaneko, M. Moriguchi, Y. Koseki, H. Kajiyama, N. Kamatani, S. Tiitinen, K. Kaarela, M. Hurme, C. P. J. Maury

chapter |3 pages

A Prospective Analysis of Demography, Etiology, and Clincial Findings of AA Amyloidosis Patients Enrolled in the International Clinical Phase II/III Fibrillex™ Study

ByW. Hauck, L.M. Dember, P.N. Hawkins, B.P.C. Hazenberg, M. Skinner, M.-C. Bouwmeester, R. Briand, E. Chicoine, C. Gurbindo, L. Hughes, D. Garceau

chapter |3 pages

TNFRSF1A Mutations in Italian Patients Affected by Apparently Sporadic Periodic Fever Syndrome

ByL. Obici, S. Marciano, G. Palladini, F. Lavatelli, S. Donadei, A. Cigni, A.E. Satta, L. Praderio, M. Tresoldi, G. Merlini

chapter |3 pages

Cathepsin B Generates Nine Different AA Amyloid Proteins by its Carboxypeptidase Acitvity

ByC. Röcken, R. Menard, F. Bühling, J. Raynes, T. Kähne

chapter |3 pages

A Serum Amyloid a Peptide Contains a Low-Ph Heparin Binding Site which Promotes AA-Amyloidogenesis in Cell Culture

ByElena Elimova, Robert Kisilevsky, John B. Ancsin

chapter |3 pages

Analyses of the Amyloid Fibrils in Bovine Amyloidosis in Hokkaido District, Japan

ByM. Ueda, Y. Ando, K. Haraoka, T. Matsui, N. Takamune, S. Saito, M. Nakamura, H. Terazaki, T. Yamashita, S. Xuguo, J. Kim, T. Tateishi, S. Shoji, Y. Hoshii, T. Ishihara, M. Otagiri, M. Uchino

chapter |3 pages

Serum Amyloid a (SAA) in Mammary Tissues with Inflammatory Processes and in Mammary Corpora Amylacea

ByM.J.M. Toussaint, C. Hogarth, T.K.A. Nguyen, E. Loeb, J. Balciute, V. Vivanco, A.M. van Ederen, S. Jacobsen, T.A. Niewold, E. Gruys

chapter |3 pages

Chemical Typing of Porcine Systemic Amyloid as AA-Amyloid

ByT.A. Niewold, C.L. Murphy, M.J.M. Toussaint, E. Gruys

chapter |3 pages

Serum Amyloid P Component in Mink *

ByLone A. Omtvedt, Tale N. Wien, Theresa Myran, Knut Sletten, Gunnar Husby

chapter |3 pages

Acute-Phase Serum Amyloid a (A-SAA) Synthesized by Human Synovial Cells (SC) Induces SC Migration

ByY. Kumon, K. Arii, T. Suehiro, Y. Ikeda, H. Enzan, K. Hashimoto

chapter |3 pages

Serum Amyloid a (SAA) Induces Cytokine Production in Human Mast Cells

ByK. Niemi, M.H. Baumann, K.K. Eklund

chapter |2 pages

Agrin is a Component of AA Amyloid

ByT.N. Wien, R. Sørby, T. Landsverk, G. Husby

chapter |2 pages

Autofluorescence of AA Amyloid

ByT.N. Wien, R. Sørby, T. Landsverk, G. Husby

chapter |3 pages

Fibrillar Proteins Occuring in the Nature Can Induce AA Amyloidosis in Mice

ByK. Lundmark, G.T. Wustermark, A. Olsén, P. Westermark

chapter |3 pages

Amyloid Production by Human Monocyte Cultures

ByB. Kluve-Beckerman, N. Magy, J. J. Liepnieks, M. D. Benson

chapter |4 pages

Experimental Amyloidosis Induced by Freund’s Adjuvant and Casein in Two Mouse Strains

ByR. Bradunaite, D. Povilenaite, L. Leonaviciene, D. Vaitkiene, A. Venalis

chapter |3 pages

Fibrin and Ubiquitin Enhance the Development of Casein-Induced Experimental AA Amyloidosis in C57BL/6 Mice

ByL. Leonaviciene, D. Povilenaite, R. Bradunaite, D. Vaitkiene, A. Venalis

chapter |3 pages

Lower Propensity for Amyloidogenesis in Female Mice

ByS. Shtrasburg, M. Pras, M. Dulitzky, C. Pariente, R. Gal, A. Mor, A. Livneh

chapter |3 pages

Systemic AA Amyloidosis in the Common Marmoset

ByE. Ludlage, C. L. Murphy, S. M. Davern, A. Solomon, D.T. Weiss, D. Glenn-Smith, S. Dworkin, K. G. Mansfield

chapter |3 pages

The Inhibitory Effect of Monensin on Murine AA Amyloidosis

ByT. Gondo, D. Cui, Y. Hoshii, H. Kawano, M. Takahashi, T. Iwata, T. Ishihara

chapter |3 pages

Amyloid-Determining Residues in Mouse Serum Amyloid A

ByB. Kluve-Beckerman, D. Marks, L. Wang, M. D. Benson, J.J. Liepnieks

chapter |2 pages

Hydrocortisone Suppresses the Second Phase of Amyloidogenesis in a Mouse Model

ByS. Shtrasburg, M. Pras, C. Pariente, R. Gal, A. Livneh

chapter |2 pages

Targeting Apolipoprotein E Fragments in Amyloid Deposit; a Study on a Mouse Model

ByTomonosuke Someya, Toshiyuki Yamada, Mariko Kobayashi, Shinobu Fujita

chapter |3 pages

Scintigraphy at Different Time Intervals after Administration of 123I Labelled Serum Amyloid P Component (SAP) in Patients with Amyloidosis

ByB.P.C. Hazenberg, P.L. Jager, D.A. Piers, P.C. Limburg, M.N. de Hooge, M.H. van Rijswijk

chapter |3 pages

Patient Information in AA Amyloidosis - What the Patients Want

ByJ.A. Hunter, E. Ellison, A. Francks, M. Gupta, R. Swann, N. Hanif

chapter |3 pages

Advanced Glycation End Products and Advanced Oxidation Protein Production in Patients with AA Amyloidosis

ByR. Ryšavá, M. Kalousová, T. Zima, C. Dostál, M. Merta, V. Tesař

chapter |3 pages

Epidemiologic Description of Amyloidosis Diagnosed in University Hospital of Rennes From 1995 to 1999

ByC. Cazalets, B. Cador, N. Mauduit, O. Decaux, M.P. Ramée, P. Le Pogamp, M. Laurent, P. Jego, B. Grosbois

chapter |3 pages

A Heterozygotic Japanese Patient with Familial Mediterranean Fever (FMF) Pyrin M694I

ByH. Terazaki, A. Okuda, K. Katase, Nakamura Mi, M. Ueda, Kim Jm, K. Haraoka, Y. Ando, Y. Sasaki

chapter |3 pages

Comparison of Clinical and Immunohistochemical Findings in Patients with Rheumatoid Arthritis and Secondary Amyloidosis

ByD. Povilenaite, V. Graziene, I. Butrimiene, A. Venalis

chapter |3 pages

Renal Glomerular AL and Vascular AA Amyloidosis in a Patient with Ankylosing Spondylitis

ByT. Pettersson, P. Anttila, T. Törnroth

chapter |2 pages

Fat Tissue Analysis by Congo Red Method and by Amyloid a Protein Qualification in Clinical AA Amyloidosis

ByB.P.C. Hazenberg, J. Bijzet, P.C. Limburg, M.H. van Rijswijk, D. Garceau

chapter |3 pages

Influence of Inflammation on Lipid Metabolism in the Patients with Rheumatoid Arthritis

ByY. Koseki, C. Terai, H. Kajiyama, M. Ito, H. Yamanaka, M. Hara, T. Tomatsu, N. Kamatani

section Section 4|76 pages

Familial Amyloidosis

chapter |3 pages

Molecular and Cellular Aspects in Transthyretin Amyloidosis

ByM.J. Saraiva

chapter |3 pages

The Binding of Diethylstilbestrol to Transthyretin – A Crystallographic Model

ByE. Morais-de-Sá, P.J.B. Pereira, M.J. Saraiva, A.M. Damas

chapter |3 pages

Liver Transplantation in ATTR Met30 and Other Variants: Time to Shift the Paradigm?

ByA.J. Stangou, N.D. Heaton, M. Rela, J. O’Grady, C.J. Mathias, H.J.B. Goodman, H.J. Lachmann, A. Bybee, D. Rowzcenio, J. Joshi, R.S. Williams, M.B. Pepys, P.N. Hawkins

chapter |3 pages

Non-Transthyretin Hereditary Amyloidoses

ByMerrill D. Benson

chapter |3 pages

Fibrinogen a α-Chain Amyloidosis: Clinical Features and Outcome after Hepatorenal or Solitary Kindey Transplantation

ByA.J. Stangou, H.J. Lachmann, H.J.B. Goodman, A. Bybee, D. Rowzcenio, G. Tennent, S.O. Brennan, J.G. O’Grady, N.D. Heaton, M. Rela, M.B. Pepys, P.N. Hawkins

chapter |1 pages

A Novel Complex Indel Mutation in the Fibrinogen Aα Chain Gene in an Asian Child with Systemic Amyloidosis

ByA. Bybee, H.G. Kang, I.S. Ha, M.S. Park, H.I. Cheong, Y. Choi, J.A. Gilbertson, M.B. Pepys, P.N. Hawkins

chapter |1 pages

Phenotype, Genotype and Outcome in Hereditary ApoAI Amyloidosis

ByP.N. Hawkins, A. Bybee, H.J.B. Goodman, H.J. Lachmann, D. Rowczenio, J.A. Gilbertson, J. O’Grady, N. Heaton, A. Stangou, M.B. Pepys

chapter |3 pages

Pigmented Ciliary Ephithelium Cells Synthesized Transthyretin in the Rabbit Eye

ByT. Kawaji, Y. Ando, M. Nakamura, K. Yamamoto, E. Ando, M. Fukushima, A. Hirata, H. Tanihara

chapter |3 pages

The X-Ray Crystallographic Structure of the Amyloidogenic Variant TTR TYR78PHE

ByR. Neto-da-Silva, P. J. B. Pereira, M. J. M. Saraiva, A. M. Damas

chapter |3 pages

An Interactive Strategy for the Accurate Characterization of Transthyretin Variants Using Combined Proteomic and Genomic Technologies

ByS.R. Zeldenrust, H.R. Bergen, A.I. Nepomuceno, M.L. Butz, D.S. Snow, P.J. Dyck, P.J.B. Dyck, C.J. Klein, J.F. O’Brien, S.N. Thibodeau, D.C. Muddiman

chapter |3 pages

Macroscopic Assessment of Skin Venules in Patients with Familial Amyloidotic Polyneuropathy (FAP) Using Near-Infrared Spectrophotoscopy: As a Marker of Autonomic Dysfunction

ByK. Obayashi, Y. Ando, T. Miida, M. Nakamura, T. Yamashita, M. Ueda, K. Haraoka, H. Terazaki, M. Okada

chapter |2 pages

Incidence of Transthyretin VAL122ILE Amyloid Mutation in African-Americans Born in Indianapolis, Indiana, USA

ByM.D. Benson, M. Yazaki, T. Yamashita, K. Hamidi Asl

chapter |3 pages

An Historical Case of Primary Amyloidosis was Re-Diagnosed as ATTR (V30M)

ByR.P. Linke, W. Schlote, L. Gerhard, P. Winter, K. Altland

chapter |3 pages

Echocardiographic Abnormalities in Transthyretin (TTR) VI22I Allele Carriers

ByC.E. Tagoe, A. Schwartzbard, A. Shah, J.N. Buxbaum, L. Salman, E. Feliciano, M. Labat, D. Mehra, S. Malendowicz, G. Hanna, S. Pierre, M.R. Stedman, R. Roswell, L.D. Flore, M.T. Brophy, D. Gagnon, D.R. Jacobson

chapter |3 pages

Familial Amyloidotic Polyneuropathy with Severe Renal Involvement in Association with Transthyretin Gly47Glu in Dutch, British and American Families

ByE.B. Haagsma, P.N. Hawkins, M.D. Benson, H.J. Lachmann, A. Bybee, B.P.C. Hazenberg

chapter |3 pages

Clinical Diagnosis of Familial Transthyretin Amyloidosis by Automated Data Acquisition and Processing with Quadrupole Orthogonal Time-of-Flight Mass Spectrometry

ByM.E. Mccomb, A. Lim, R. Théberge, T. Prokaeva, L.H. Connors, M. Skinner, C.E. Costello

chapter |3 pages

Liver Transplantation for Familial Amyloidotic Polyneuropathy Does Not Prevent Disease Progression in a Majority of Patients

ByS. Zeldenrust, R. Perri, D. Brandhagen, C. Rosen, J. Poterucha, M. Gertz, R. Wiesner, G. Gores

chapter |3 pages

Continued Deposition of Wild-Type Transthyretin in Cardiac Amyloid after Liver Transplantation

ByJ.J. Liepnieks, M. Yazaki, M.D. Benson

chapter |3 pages

Deposition and Passage of Transthyretin Through the Blood-Nerve Barrier in Recipients of Familial Amyloid Polyneuropathy Livers

ByM. M. Sousa, J. Ferrão, R. Fernandes, A. Guimarães, J. B. Geraldes, R. Perdigoto, L. Tomé, O. Mota, L. Negrão, A. L. Furtado, M. J. Saraiva

chapter |3 pages

Familial Amyloidosis: Recent Novel and Rare Mutations in a Clinical Population

ByL.H. Connors, T. Prokaeva, H. Akar, M. Metayer, P. Smith, A. Lim, R. Théberge, C.E. Costello, M. Skinner

chapter |3 pages

Renal Apolipoprotein A-I Amyloidosis (Aapo-A-I) Associated with a Novel Mutant Leu64Pro

ByC. L. Murphy, S. Wang, K. Weaver, M.A. Gertz, D.T. Weiss, A. Solomon

chapter |1 pages

Hereditary Amyloidosis in a Spanish Family Associated with a Novel Nonstop Mutation in the Gene for Apolipoprotein AII

ByD. Rowczenio, J.A. Gilbertson, A. Bybee, D. Hernández, Hawkins Pn

chapter |1 pages

Hereditary Renal Amyloidosis in a German Family Associated with Fibrinogen a α Chain Glu540Val

ByA. Bybee, M. Hollenbeck, E.R. Debusmann, D. Gopau, J.A. Gilbertson, H.J. Lachmann, M.B. Pepys, P.N. Hawkins

chapter |3 pages

Systemic ALys Amyloidosis Presenting with Gastrointestinal Bleeding

ByC. Röcken, K. Becker, B. Stix, T. Rath, T. Kähne, M. Fändrich, A. Roessner, F.W. Albert

section Section 5|42 pages

Central Nervous System Amyloidosis

chapter |3 pages

Prion Diseases and the Prion Protein

ByPierre Aucouturier, Etienne Levavasseur, Clara Ballerini, Claude Carnaud

chapter |3 pages

Exploring the Folding and Aggregation Mechanisms of Amyloid-Forming Peptides by Computer Simulations

BySébastien. Santini, Guang-Hong Wei, Normand Mousseau, Philippe Derreumaux

chapter |3 pages

Camp-Specific Pde4B Mediates Aβ- Induced Microglial Activation

ByG. Sebastiani, C. Morissette, C. Lagacé, M. Boulé, M.J. Ouellette, R.W. Mclaughlin, D. Lacombe, F. Gervais, P. Tremblay

chapter |3 pages

Perspectives of Immunotherapy Against TSE

ByC. Carnaud, S. Gregoire, F. Chatelet, M. Bruley-Rosset, P. Aucouturier

chapter |3 pages

Serpinopathies and the Central Nervous System

ByDavid A. Lomas, Didier Belorgey, Meera Mallya, Maki Onda, Kerri J. Kinghorn, Lynda K. Sharp, Russell L. Phillips, Richard Page, Elena Miranda, Damian C. Crowther

chapter |2 pages

Hereditary Cerebral Amyloid Angiopathy Associated with ATTR Tyr114Cys

ByT. Yamashita, Y. Ando, M. Nakamura, K. Haraoka, S. Xuguo, H. Terazaki, M. Ueda, T. Ikeda, S. Saito, T. Kawaji, T. Hirai, Y. Washimi, M. Uchino

chapter |3 pages

Hereditary Cerebral Amyloid Angiopathy Associated with a Novel Amyloid Beta Precursor Protein Mutation

ByL. Obici, G. De Rosa, G. Palladini, S. Marciano, S. Donadei, E. Arbustini, L. Verga, M. Concardi, G. Ferrari, G. Merlini

chapter |3 pages

Cognitive Disturbance and Brain Pathology in Old Dogs

ByJ. Rofina, M.J.M. Toussaint, A.M. Van Ederen, M. Secrève, E. Gruys

chapter |3 pages

D-Amino-Acid Peptide Specific for Cerebral Aβ-Amyloid in Man Cross-Reacts with Canine Cerebral Amyloid

ByR.P. Linke, R. Oos, J.E. Rofina, K. Wiesehan, D. Willbold, E. Gruys

chapter |3 pages

Inhibition of ß-Amyloid Peptide-Induced Microglial Cell Activation by Glycosaminoglycans

ByC. Morissette, F. Gervais, P. Tremblay

chapter |2 pages

Reversing the Formation of Alzheimer Amyloid Fibrils and Blocking Toxic Ca2+ Influx

ByV.M. Ingram, B.J. Blanchard, A. Chen, K. Stafford, B. Stockwell

section Section 6|64 pages

Localized Amyloidosis

chapter |3 pages

β2 Microglobulin Amyloidosis

ByA. Argilés, A. Charnet, C.F. Schmitt-Bernard, M. García-García, C. Méjean, G. Mourad

chapter |3 pages

Amyloid Tumors of Breast, Lung and RIB in Nonsecretory Multiple Myeloma

ByS. Richter, G. Sauter, L. Jonas, E. Gafumbegete, J. Makovitzky

chapter |3 pages

The Spectrum of Localized Amyloidosis: A Case Series of Twenty Patients

ByMl Biewend, Dm Menke, Kt Calamia

chapter |2 pages

Localized Amyloidosis: About 36 Cases

ByM. Paccalin, M. Rubi, E. Hachulla, M. Carreiro, L. Tricot, B. Wechsler, J. Cabane, C. Cazalet, P. Roblot

chapter |3 pages

Studies on Unfolded β2-Microglobulin C-Terminal 92 to 99 in Dialysis Related Amyloidosis

ByY. Motomiya, K. Haraoka, X. Sun, H. Monta, Y. Ando

chapter |3 pages

Kappa III Immunoglobulin Light Chain Origin of Localized Orbital Amyloidosis

ByK. Hamidi Asl, J.J. Liepnieks, W.R. Nunery, M. Yazaki, M.D. Benson

chapter |2 pages

Corneal Dystrophy Caused by a Novel Mutation of the TGFBI Gene: A Case Report

ByB. Stix, J. Rüschoff, A. Roessner, C. Röcken

chapter |3 pages

Medullary Thyroid Carcinoma-Associated Amyloid is Comprised of Calcitonin Gene-Related Peptide II

ByC. L. Murphy, S. Wang, B. Crombie, S.D. Macy, T.K. Williams, D.T. Weiss, A. Solomon

chapter |3 pages

Studies of the Amyloidogenic Properties of proIAPP

ByJ. F. Paulsson, G. T. Wustermark

chapter |2 pages

Laryngeal Amyloidosis: Localized Versus Systemic Disease

ByH. Bartels, F.G. Dikkers, J.E. Van Der Wal, H.M. Lokhorst, B.P.C. Hazenberg

chapter |3 pages

Formation of the Vascular Amyloid Fibril Protein Medin From its Precursor Lactadherin

ByS. Peng, A. Persson, E. Wassberg, P. Westermark

chapter |3 pages

Effects of Medin on Smooth Muscle Cells

ByA. Persson, S. Peng, P. Gerwins, X. Fu, P. Westermark

chapter |3 pages

Transmission of Mouse Aapoaii Amyloidosis by the Amyloid Fibrils; Inhibitory Effects on Transmission by the Denaturation/ Degradation of Amyloid Fibrils

ByJinko Sawashita, Huanyu Zhang, Fu Xiaoying, Tatsumi Korenaga, Masayuki Mori, Keiichi Higuchi

chapter |3 pages

Transmission of Mouse Aapoaii Amyloidosis from Mother to Pups

ByT. Korenaga, X. Fu, M. Mori, J. Sawashita, H. Naiki, T. Matsushita, K. Higuchi

chapter |3 pages

External Beam Radiation for Tracheobronchial Amyloidosis: The Boston University Experience

ByJ.L. Berk, G.A. Grillone, M. Skinner, A.C. Hartford

chapter |3 pages

Pindborg Tumor-Associated Amyloid Proteins

ByC. L. Murphy, S. Wang, D.P. Kestler, S.D. Macy, T.K. Williams, E.R. Carlson, D.T. Weiss, A. Solomon

chapter |3 pages

Semenogelin I is the Amyloidogenic Protein in Senile Seminal Vesicles

ByR.P. Linke, R. Joswig, C.L. Murphy, S. Wang, H. Zhou, U. Gross, C.B. Röcken, W. B.J. Nathrath, P. Westermark, D. Weiss, A. Solomon

chapter |3 pages

Cardiac Disease and Heart Valve Amyloid: What is the Connection?

ByStina Enqvist, Erik Wassberg, Malin Wilhelmsson, Ulf Hellman, Per Westermark

section Section 7|36 pages

Therapeutics

chapter |3 pages

Immunotherapy of AL Amyloidosis

ByAlan Solomon, Deborah T. Weiss, Jonathan S. Wall

chapter |3 pages

The Amyloid-Reactive Monoclonal Anitbody 11-1F4 Binds a Cryptic Epitope on Fibrils and Partially Denatured Immunoglobulin Light Chains and Inhibits Fibrillogenesis

ByB. O’Nuallain, C.L. Murphy, D.A. Wolfenbarger, S. Kennel, A. Solomon, J.S. Wall

chapter |3 pages

Glycosaminoglycans as a therapeutic target of amyloid disorders

ByB.P.C. Hazenberg

chapter |3 pages

Effects of a New Diflunisal Derivative on Transthyretin Binding and Stabilization in Serum from FAP Patients

ByM. R. Almeida, B. Macedo, I. Cardoso, G. Valencia, G. Arsequell, A. Planas, M. J. Saraiva

chapter |3 pages

Suppression of Transthyretin Synthesis by Antisense Oligonucleotides

ByM.D. Benson, B. Kluve-Beckerman, K.W. Sloop, D.M. Bodenmiller

chapter |2 pages

Targeted Conversion of the Transthyretin Gene In Vitro and In Vivo

ByM. Nakamura, Y. Ando, M. Ueda, T. Kawaji, T. Yamashita, J.M. Kim, K. Haraoka, H. Terazaki, S. Nagahara, A. Sano, S. Saito, M. Uchino

chapter |3 pages

Identification of New Diflunisal Derivatives as Potent In Vitro Transthyretin Fibril Inhibitors

ByT. Mairal, G. Arsequell, G. Valencia, I. Dolado, J. Nieto, A. Planas, J. Barluenga, A. Ballesteros, R. Almeida, M.J. Saraiva

chapter |2 pages

The Future of Amyloidosis

ByMerrill D. Benson

section Section 8|14 pages

Satellite Symposium Neurochem Inc.

chapter |4 pages

The Changing Face of AA Amyloidosis

ByB.P.C. Hazenberg

chapter |3 pages

Uncommon Conditions Underlying AA Amyloidosis

ByG. Merlini

chapter |3 pages

Therapeutic Management of AA Amyloidosis: From Bench to Bedside

ByMartha Skinner

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