1st Edition

XIth International Symposium on Amyloidosis

    432 Pages 174 B/W Illustrations
    by CRC Press

    Edited by a stellar team of scientists compiling more than 120 papers into a single volume, the XIth Annual International Symposium on Amyloidosis represents the most important review of the state-of-the-science and future directions of this rapidly growing field. An unparalleled cast of pioneering researchers, including keynote speaker and Nobel Laureate Dr. Stanley Prusiner, present groundbreaking research in systemic amyloidosis including the mechanisms of disease and cellular toxicity, AA amyloidosis, familial amyloidosis, AL amyloidosis, clinical trials, and emerging Divided into six sections, the book begins with a study of the mechanisms of fibril formation and cellular response. It investigates possible horizontal transmission, charge differences of deposits in AL and NALCDD, and novel assays to detect amyloid induced apoptosis. The second section considers AA and localized amyloidosis and presents advances in non-invasive imaging, amyloid typing, and the translocation of systemic amyloidosis. The section on familial and TTR amyloidosis features an original report on hereditary gelsolin amyloidosis in an Iranian family, as well as other novel and rare mutations. An extensive section on AL amyloidosis introduces ALBASE as a platform analysis tool, AL amyloidosis in the elderly, and a study of the therapeutic potential of naturally occurring antibodies. Section five focuses on new and emerging diagnostic and translational approaches including characterization and identification through biopsy, mass spectrometry, proteomics, and the use of an amyloid "chip". The book concludes with clinical trials of diflunisal and high dose melphalan-dexamethasone and autologous stem cell transplantation.
    translational approaches.


    Focusing on new basic and translational medicine approaches in systemic amyloidosis, XIth Annual International Symposium on Amyloidosis provides clinicians and researchers with an invaluable single source reference to the most up-to-date research in the field.

    Preface
    Outstanding Achievement Award - Dr. Alan S. Cohen
    Symposium Keynote Address - Dr. Stanley Prusiner
    Report of the Nomenclature Committee


    MECHANISMS OF FIBRIL FORMATION AND CELLULAR RESPONSE
    KEYNOTE ADDRESS: The Effect of Membranes and Surfaces on the Fibrillation of an Amyloidogenic Light Chain: Relevance to the Etiology of Al Amyloidosis, X. Meng, M. Zhu, and A.L. Fink

    Heparan Sulfate Promotes the Refolding and Oligomerization of SAA1.1: A Short Sequence is Implicated in this Misfolding Process, J.B. Ancsin, E. Elimova, and R. Kisilevsky

    Mouse Senile Amyloidosis: Possible Horizontal Transmission in the Mouse Room, X. Fu, T. Korenaga, J. Yan, F. Ge, B. Zhang, J. Qian, H. Naiki, J. Sawashita, M. Mori, and K. Higuchi

    Charge Differences of Deposits in AL and NALCDD, B. Kaplan, A. Livneh, and G. Gallo

    Role of the C-terminal Amino Acids in ß2-Microglobulin Amyloid Formation, J. Kim, Y. Motomiya, M. Nakamura, M. Ueda, S. Saito, Y. Misumi, S. Himeno, K. Obayashi, S. Shinriki, W. Meng, U. Semba, H. Kai, and Y. Ando

    Serum Amyloid A Generates High-Density Lipoprotein from Human Aortic Smooth Muscle Cells, Y. Kumon, S. Abe-Dohmae, S. Yokoyama, and T. Sugiura

    Amyloid Light Chains Localize to Lysosomes and Cardiac Fibroblasts Show Reduced Mitochondrial Function, G. Monis, J. Eberhard, L.H. Connors, M. Skinner, and V. Trinkaus-Randall

    Cerebrospinal Fluid and Plasma of Alzheimer Patients Promote ß-Amyloid Fibril Formation In Vitro, K. Ono, M. Noguchi-Shinohara, M. Samuraki, Y. Matsumoto, D. Yanase, K. Iwasa, H. Naiki, and M. Yamada

    A Novel Assay for Detection of Amyloid Induced Apoptosis, J.F. Paulsson, S.W. Schultz, M. Köhler, I. Leibiger, P.-O. Berggren, and G.T. Westermark

    Human Amyloidogenic Immunoglobulin Light Chains Alter Cardiomyocyte Signaling and Disrupt Heparan Sulfate Proteoglycan (HSPG) Expression, X. Peng, E. Kim, T. Miller, G. Karamitis, L.H. Connors, M. Skinner, and D.B. Sawyer

    GAGs Associated with Fibril Extracts Mediate Fibrillogenesis in AL Amyloidosis, R. Ren, Z. Hong, L.H. Connors, M. Skinner, C.E. Costello, and V. Trinkaus-Randall

    Decorin in AA Amyloidosis, T.N. Wien, R. Sørby, T. Landsverk, and G. Husby

    Structural Evolution of Immunoglobulin Light Chain during Amyloid Fibril Formation: A Deep UV Resonance Raman Spectroscopic Study, M. Xu, E.M. Baden, K.M. Jasmin, M. Ramirez-Alvarado, and I.K. Lednev


    AA AND LOCALIZED AMYLOIDOSES
    KEYNOTE ADDRESS: AA Amyloidogenesis: Basic and Translational Research, R. Kisilevsky and W.A. Szarek


    Histochemical and Immunohistochemical Characteristics of Amyloid Deposits, Á. Apáthy and M. Bély

    Ultrastructural Characteristics of Amyloid Deposits, M. Bély, Á. Apáthy, and P. Kapp

    Odontogenic Feline and Canine Tumor-Associated Amyloid is Formed from Ameloblastin, R. Donnell, C.L. Murphy, M. Eulitz, T.K. Williams, S.D. Macy, D.T. Weiss, and A. Solomon

    Amyloidogenic Region of Odontogenic Ameloblast Associated Protein (ODAM), J.S. Foster, D.P. Kestler, C.L. Murphy, S.J. Kennel, J.S. Wall, D.T. Weiss, and A. Solomon

    What is the Best Way to Assess Predisposition to AA Amyloid in Inflammatory Joint Disease?, J.A. Hunter, R.D. Baxter, A. Stirling, J. France, F. Varyani, and M.S. Mak

    Advances in Non-Invasive Imaging of AA Amyloid in Mice, S.J. Kennel, T. Richey, J.S. Avenell, P.N. Hawkins, A. Solomon, and J.S. Wall

    Detection and Identification of Amyloid Deposition Associated with Human Unerrupted Tooth Follicles, D.P. Kestler, J. Hudson, S.D. Macy, C.L. Murphy, J.S. Foster, D.T. Weiss, and
    A. Solomon

    Vascular AA Amyloid Formation Modeled in Cell Culture, B. Kluve-Beckerman

    Amyloidosis in Ankylosing Spondylitis - Unexpected Findings Underscoring the Importance of Typing of Amyloid Deposits, D. Kracker, N.O. Litbarg, and M.M. Picken

    Lack of Correlation Between Amyloid A Protein Levels and Clinical Parameters in Aspirated Fat Tissue of Familial Mediterranean Fever Patients with Secondary Amyloidosis, M. Lidar, B.P. Hazenberg, J. Bijzet, W. Hauck, D. Garceau, R. Kedem, and A. Livneh

    Chronic, Slowly Progressing Nephropathy Due to Amyloidosis of CSAA (SAA4) Origin, R.P. Linke, S. Adler, U. Hegenbart, S. Schönland, J. Lutz, F.C. deBeer, and H.-J. Gröne

    Redistribution, Clearance and Re-induction of Amyloid in Reactive Murine AA Amyloidosis, S.N. Nyström and G.T. Westermark

    N-Formyl Peptide Receptor-Like 1 of the Neutrophils in the AA-Amyloidosis Tissues is Less Stained, E. Ohara, Y. Kumon, and T. Sugiura

    AL in the Clinical Setting of AA: The Importance of Amyloid Typing, K. Oshima, D. Kracker, and M.M. Picken

    Foie Gras is a Dietary Source of AEF, T. Richey, C.L. Murphy, J.S. Wall, G.T. Westermark, P. Westermark, D.T. Weiss, and A. Solomon

    The Effect of a Variety of Anti-Inflammatory Drugs on Murine Amyloidogenesis, S. Shtrasburg, M. Lidar, M. Pras, R. Gal, and A. Livneh

    Translocation of Systemic Amyloidosis by Peripheral Blood Monocytes, J. Sponarova, S.N. Nystrom, and G.W. Westermark

    FK506 Inhibits Murine AA Amyloidosis Without Modulating Serum SAA Levels, M. Ueda, Y. Ando, M. Nakamura, T. Yamashita, K. Obayashi, Y. Misumi, S. Shinriki, A. Ueda, R. Zeledon, S. Himeno, S. Saito, J. Kim, W. Meng, and M. Uchino

    Local Tumorous AA-Amyloid Deposition in a Case of Hepatic Adenomatosis: Immunohistochemistry and In Situ Hybridization Employing SAA-mRNA Probes, S. Urieli-Shoval, P. Flemming, M. Stolte, and R.P. Linke

    SAA4-Related AA Amyloidosis, S. Wang, C.L. Murphy, D. Kestler, S.D. Macy, T.K. Williams, D.T. Weiss, and A. Solomon

    Measurement of Amyloidogenic SAA Isotype, SAA1, T. Yamada, and Y. Okuda


    FAMILIAL AND TTR AMYLOIDOSES
    In Vitro and In Vivo Effects of Genistein on TTR Stabilization and Aggregation, M.R. Almeida, I. Cardoso, N. Ferreira, and M.J. Saraiva

    Labial Salivary Gland Biopsy for Diagnosis of Amyloid Polyneuropathy Portuguese Type, B. Amaral, T. Coelho, and A. Guimarães

    Hereditary Gelsolin Amyloidosis in an Iranian Family: The First Report from the Middle East, M.R. Ardalan, M.M. Shoja, T. Paunio, S. Tanskanen, S. Kiuru-Enari, A. Rastegar, and M. Kashgarian

    A Case of Compound Heterozygosity in a Nigerian Male with Identification of a New Transthyretin Variant Phe44Leu and Val122Ile, C.T. Barry, T. Prokaeva, P. Soohoo, B. Spencer, D. Pimental, and L.H. Connors

    Suppression of HepaticT synthesis by Antisense Oligonucleotides, M.D. Benson and B. Kluve-Beckerman



    Top-Down Mass Spectrometry Analysis of Amyloidogenic Proteins, C.E. Costello, R. Théberge, C. Lin, J. Kingsbury, E. Klimtchuk, L.H. Connors, M. Skinner, D.C. Seldin, and P.B. O'Connor

    ATTR (Thr59Lys) Amyloidosis with Involvement of Heart, Peripheral and Autonomic Nervous System, Gut and Salivary Glands in an Egyptian Man, Previously Diagnosed as "Familial Amyloid Neuropathy of Portuguese Type", G.E. Feurle, J.J. Liepnieks, M.D. Benson, and R.P. Linke

    Systemic Amyloidosis of Fibrinogen Origin in the Wild Living Stone Marten (Martes Foina), O. Geisel, R. Mentele, F. Lottspeich, and R.P. Linke

    Familial Amyloid Nephropathy of Fibrinogen Aa (E526V) Origin. Report of the First Case from Austria, S. Hargassner, G. Biesenbach, G. Syré, K. Altland, and R.P. Linke

    Laryngeal Amyloidosis in Patients with Apolipoprotein AI I174S and I178P, A.J.C. Hazenberg, F.G. Dikkers, P.N. Hawkins, J. Bijzet, D. Rowczenio, M.D. Posthumus, M.K. Leijsma, J. van der Wal, and B.P.C. Hazenberg

    Hereditary Fibrinogen A a-Chain Amyloidosis: Clinical Features and the Curative Role of Liver Transplantation, N.D. Heaton, J. O'Grady, M. Rela, P. Muiesan, J.A. Wendon, L. Sizer, J. Sedgwick, M. Thoma, F. Murgatroy, C.J. Mathias, H.J. Goodman, D. Rowczenio, A. Bybee, G. Tennent, P.N. Hawkins, and A.J. Stangou

    Amyloidosis in the Transgenic Mice of Mouse Amyloidogenic Apolipoprotein A-II (apoa2c), K. Higuchi, F. Ge, X. Fu, J. Yao, Z. Guo, J. Yan, B. Zhang, H. Zhang, J. Qian, J. Sawashita, and M. Mori

    Urinary Transthyretin: A Possible Biochemical Marker in the Early Stage of Renal Dysfunction, S. Himeno, M. Ueda, M. Nakamura, T. Yamashita, K. Obayashi, X. Sun, Y. Misumi,
    S. Shinriki, A. Ueda, Y. Uji, S. Saito, J. Kim, W. Meng, and Y. Ando

    Unique Phenotype of Vitreous Amyloidosis in Different Endemic Areas, N. Kakizoe, Y. Ando, T. Kawaji, E. Ando, O. Sandgren, O.B. Suhr, and H. Tanihara

    Composition of Peripheral Nerve Amyloid after Orthotopic Liver Transplant for FAP, J.C. Kincaid, J.J. Liepnieks, and M.D. Benson

    Characterization of Fibrillar Transthyretin in Ex Vivo Cardiac Deposits from Cases of Senile Systemic Amyloidosis, J.S. Kingsbury, R. Théberge, C.E. Costello, and L.H. Connors

    Comparison of Cardiac Amyloidosis Associated with Wild Type Transthyretin and the Isoleucine 122 Variant, H.J. Lachmann, H.J.B. Goodman, J.D. Gillmore, A. Wechalekar, D.M. Rowczenio, A. Bybee, and P.N. Hawkins

    AL Amyloidosis in a Patient with a T60A TTR Mutation, H.J. Landau, R.L. Comenzo, P. Zhou, B. Clark, J. Teruya-Feldstein, S. Wang, C.L. Murphy, and A. Solomon

    Biochemical Characterization of Leptomeningeal Amyloid in a Val30Gly Transthyretin Patient, J.J. Liepnieks, S. Vakili, and M.D. Benson

    Apolipoprotein A-I (apo A-I) Nature of Knee Joint Meniscus-Associated Amyloid, S.D. Macy, T.K. Williams, C. Wooliver, C.L. Murphy, S. Wang, D. Kestler, P. Westermark, D.T. Weiss, and A. Solomon

    An Autopsy Case of Familial Amyloidotic Polyneuropathy ATTR Val30Met 10 Years After Liver Transplantation: Changes in the Amount of Amyloid Deposits in the Pancreas, Y. Misumi, Y. Ando, M. Nakamura, T. Yamashita, S. Himeno, M. Ueda, X. Obayashi, X. Sun, S. Saito, J. Kim, S. Shinrik, W. Meng, A. Ueda, and M. Uchino

    Amyloid Neuropathy in a Japanese Domino Liver-Transplanted Recipient, K. Obayashi, T. Yamashita, M. Ueda, M. Nakamura, K. Asonuma, Y. Inomata, M. Uchino, K. Tanaka, and Y. Ando

    The Natural History of Hereditary Apolipoprotein A-I Amyloidosis According to a Large Series of Italian Leu75Pro Patients, L. Obici, G. Gregorini, F. Scolari, l. Calabresi, T. Scalvini, S. Donadei, and G. Merlini

    Domino Liver Transplantation Using the Explanted Grafts from Patients with Hereditary Forms of Amyloidosis: The King's College Hospital Experience, J. O'Grady, M. Rela, P. Muiesan, M. Bowles, R. Williams, P.N. Hawkins, N.D. Heaton, and A.J. Stangou

    Cardiomyopathy in FAP Val30Met Patients With and Without Liver Transplantation, S. Okamoto, T. Yamashita, Y. Ando, and M. Uchino

    Detection of Cardiac Pre-Amyloid Deposits of Transthyretin by MALDI-TOF, R.N. Picken, M. Morgan, and M.M. Picken

    Effect of Nitric Oxide in Transthyretin-Related Amyloidosis, S. Saito, Y. Ando, M. Ueda, J. Kim, M. Nakamura, T. Yamashita, K. Obayashi, Y. Misumi, S. Shinriki, S. Himeno, W. Meng, Y. Ishima, T. Akaike, and M. Otagiri

    Inhibition of Intracellular Tetramerization Induces the Proteasomal Degradation of Amyloidogenic Transthyretin Variants, T. Sato, S. Susuki, M. Miyata, Y. Ando, T. Shuto, M.A. Suico, and H. Kai

    Structural Analyses of Fibrinogen Aa-Chain Amyloid Fibrils, L.C. Serpell, M.D. Benson, J.J. Liepnieks, and P.E. Fraser

    A Transthyretin Mutation (V32A) in an Israeli Patient with Amyloidotic Neuropathy Localizes to a 3-D Mutation Cluster, Y. Shinar, B. Kaplan, C. Avisar, and A. Livneh

    Amyloid Deposition in the Tongue of Patients with Familial Amyloidotic Polyneuropathy, S. Shinriki, M. Ueda, T. Yamashit, K. Obayashi, X. Sun, S. Himeno, A. Ueda, Y. Misumi, S. Saito, J. Kim, W. Meng, M. Shinohara, and Y. Ando

    Recent Novel and Rare Mutations in a Clinic Population of Patients with Amyloidosis, B. Spencer, L.H. Connors, T. Prokaeva, P. Soohoo, C. O'Hara, and M. Skinner

    Proliferation of Smooth Muscle Cells in the Tunica Intima of Cerebral Vessels in Familial Amyloidtic Polyneuropathy ATTR Y114C, A. Ueda, M. Ueda, T. Yamashita, T. Hirano, M. Nakamura, Y. Misumi, W. Meng, T. Ito, M. Uchino, and Y. Ando

    Reengineering TTR Amyloid Inhibition Properties of Diflunisal, M. Vilaró, G. Arsequell, G. Valencia, A. Ballesteros, J. Barluenga, J. Nieto, A. Planas, R. Almeida, and M.J. Saraiva

    Familial Amyloidosis Associated with a Novel Mutation (D68G) in the Lysozyme Gene, C. Wooliver, D. Coriu, C.L. Murphy, D.P. Kestler, S. Wang, D.T. Weiss, and A. Solomon

    Effects of Liver Transplantation on Hereditary Cerebral Amyloid Angiopathy Associated with ATTR Y114C, T. Yamashita, Y. Ando, M. Ueda, M. Nakamura, S. Okamoto, T. Hirahara, A. Ueda, Y. Misumi, K. Obayashi, and M. Uchino

    Effect of Tacrolimus and Cyclosporine on Transthyretin (TTR) Metabolism in Rats: A Long Term Analysis, R. Zeledon, Y. Ando, K. Asonuma, M. Nakamura, M. Ueda, X. Sun, J. Fujii, and Y. Inomata


    AL AMYLOIDOSIS
    KEYNOTE ADDRESS: Can AL Amyloidosis Be Cured?, D.C. Seldin

    Identification of Novel Inhibitors of Light Chain Fibrillogenesis, L. Acero, A. Solomon, J.S. Wall, and B. O'Nuallain

    Peptide Phage Display and Epitope Mapping of a Fibril-Related Conformational Epitope Recognized by the 11-1F4 Monoclonal Antibody, A. Allen, S.J. Kennel, A. Solomon, J.S. Wall, and B. O'Nuallain

    The Translocation t(11;14) Is Frequently Detected in Patients with AL Amyloidosis, T. Bochtler, S.O. Schonland, F. Cremer, D. Hose, M. Moos, C. Bartram, A.D. Ho, H. Goldschmidt, A. Jauch, and U. Hegenbart

    ALBASE: A Platform Analysis Tool for the Study of Amyloid Light Chain Sequence Data, K.L. Bodi, M. Kaut, B. Spencer, F. Lavatelli, C. Costello, L.H. Connors, M. Skinner, and D.C. Seldin

    Risk-Adapted Melphalan with Stem Cell Transplant (SCT) and Adjuvant Dexamethasone (DEX) +/- Thalidomide (THAL) Achieves Low Treatment-Related Mortality and High Hematologic Response Rates, A.D. Cohen, P. Zhou, L. Reich, H. Hassoun, J. Teruya-Feldstein, D.A. Filippa, B. Clark, M. Stubblefield, M. Fleisher, S.D. Nimer, and R.L. Comenzo

    A Supervised Analysis of Gene-Expression Profiles of Purified Clonal Plasma Cells From Patients with Systemic Light-Chain Amyloidosis (AL) Who Have High or Low Levels of Serum Free Lambda Light Chains, R.L. Comenzo, P. Zhou, M. Fleisher, and A.B. Olshen

    Comparison of Immunoglobulin Light Chains (?1): Amyloidogenic vs. Non-Amyloidogenic Proteins, L.H. Connors, J. Eberhard, B. Spencer, T. Prokaeva, Y. Jiang Y, M. Budnik, R. Théberge, D.C. Seldin, C.E. Costello, and M. Skinner

    Clinicopathological Analysis of Thirty-Two Patients with AL Amyloidosis: Four-Years Experience in a Single Institution, D. Coriu, C. Dobrea, M.Dogaru, S. Zamfir, E. Stoica, C. Macarie, M. Voiculescu, C. Ionescu, R. Bobeica, C. Valciu, G. Becheanu, M. Manuc, A.Tanase, S.Varady, and D. Colita

    Role of Light Chain Deamidation in the Pathogenesis of AL Amyloidosis, M. Eulitz, C.L. Murphy, D.T. Weiss, and A. Solomon

    The Extent and Distribution of Amyloid in 38 Autopsies on Patients with Primary (AL) Amyloidosis, J. Gan, L.H. Connors, M. Skinner, and C.J. O'Hara

    Transplantation without Growth Factor Engraftment Kinetics Following Stem Cell Transplantation for Primary Systemic Amyloidosis, M.A. Gertz, M Q. Lacy, A. Dispenzieri, S.R. Hayman, S.K. Kumar, and D. Gastineau

    Extent of Hematologic Response is Important in Determining Outcome in Transplanted Patients with Primary Systemic Amyloidosis. Importance of Achieving a Complete Response, S.R. Hayman, M.Q. Lacy, A. Dispenzieri, S.K. Kumar, D.A. Gastineau, and M.A. Gertz

    A Single-Center Experience Using Chemotherapy with Melphalan-Dexamethasone (M-dex) in Patients with AL Amyloidosis and Advanced Organ Involvement Not Eligible for High-Dose Melphalan Therapy (HDM), U. Hegenbart, T. Bochtler, J.B. Dengler, M. Hundemer, A.D. Ho, H. Goldschmidt, and S.O. Schonland

    Detection and Characterization of AL Light Chain Proteins from Fibrils, Z. Hong, R. Théberge, A. Lim, T. Prokaeva, L.H. Connors, M. Skinner, and C.E. Costello

    Characterization of Post-Translational Modifications of an Amyloidogenic Immunoglobulin Kappa Light Chain by Mass Spectrometry, Y. Jiang, R. Thebérge, J. Eberhard, G. Karamitis, T. Prokaeva, L.H. Connors, M. Skinner, C. Lin, P.B. O'Connor, and C.E. Costello

    Involvement of Lysosomes in Renal AL-Amyloidogenesis, J. Keeling, and G.A. Herrera

    Thermal Unfolding of Immunoglobulin Light Chains is Linked to Aggregation: Circular Dichroism and Light Scattering Studies, E.S. Klimtchuk, O. Gursky, J. Eberhard, L.H. Connors, and M. Skinner

    Prognostic Relevance of Serum N-Terminal Pronatriuretic Peptide Type B and Plasma BNP in Patients with AL Amyloidosis in Dialysis, M. Nuvolone, G. Palladini, F. Lavatelli, P. Russo, V. Perfetti, S. Perlini, L. Obici, F. Clari, and G. Merlini

    AL Amyloidosis in the Elderly: A Review of 330 Patients, M. Offer, A.D. Wechalekar, H.J. Lachmann, J.D. Gillmore, and P.N. Hawkins

    Therapeutic Potential of Naturally Occurring Fibril-Reactive Human Antibodies for AL Amyloidosis, B. O'Nuallain, A. Allen, T. Richey, J.S. Wall, D.T. Weiss, and A. Solomon

    Prolonged Follow-Up Study of AL Patients Ineligible for Stem Cell Transplantation Treated with Oral Melphalan and Dexamethasone, G. Palladini, F. Lavatelli, P. Russo, M. Nuvolone, V. Perfetti, L. Obici, and G. Merlini

    Left Ventricular Systolic Function in Patients with Cardiac AL Amyloidosis, S. Perlini, M. Vezzoli, F. Clari, G. Palladini, P. Russo, M. Nuvolone, and G. Merlini

    In Vitro Silencing of Amyloidogenic ?6 Light Chain Production, J.E. Phipps, J.S. Foster, D.P. Kestler, A. Solomon, and J.S. Wall

    Amyloid Typing in Surgical Pathology: Experience of a Single Institution, M.M. Picken

    Somatic Mutations Create Potential N-Glycosylation Sites in the Immunoglobulin Light Chain Variable Regions in Primary Amyloidosis, T. Prokaeva, B. Spencer, G. Doros, K. Bodi, J. Eberhard, Y. Jiang, M. Budnik, R. Théberge, C.E. Costello, L.H. Connors, M. Skinner, and D.C. Seldin

    A Trial of Antioxidants to Improve Cardiac Function in Patient with AL Amyloidosis and Cardiac Involvement, M. Rosenzweig, D. Sawyer, F. Ruberg, D. Wright, V. Sanchorawala, D. Seldin, J. Vita, R. Lala, D. Brenner, F. Sam, and M. Skinner

    High-Dose Melphalan Chemotherapy with Autologous Stem Cell Transplantation in 65 Patients with AL Amyloidosis: Organ Progression Can Be Delayed in Patients Reaching Complete Remission, S.O. Schonland, J.B. Dengler, M. Hundemer, T. Bochtler, R. Singer, E. Hund, J. Beimler, M. Zeier, A.V. Kristen, T.J. Dengler, A.D. Ho, H. Goldschmidt, and
    U. Hegenbart

    Glycosylated Amyloid and Non-Amyloid Associated Lambda Light Chains, K. Sletten, S. Wang, C.L. Murphy, F.J. Stevens, D.T. Weiss, and A. Solomon

    Extramedullary Production of AL Amyloid Precursor Protein, A. Solomon, D.T. Weiss, S.D. Macy, C. Wooliver, and P. Westermark

    Mesangial Amyloid Formation in an Isolated Rat Kidney Perfusion Model: A New Model to Study Renal Amyloidogenesis, J. Teng and G.A. Herrera

    Overexpression of Calreticulin in Clonal Al Plasma Cells is Associated with Response to Melphalan and Autologous Stem Cell Transplant in Systemic Light-Chain (AL) Amyloidosis, J. Teruya-Feldstein, P. Zhou, A.B. Olshen, and R.L. Comenzo

    Semi-Quantitative Assessment of Amyloid in Congo Red-Stained Subcutaneous Abdominal Fat Tissue in Systemic Amyloidosis and Disease Severity, I.I. van Gameren, B.P.C. Hazenberg, J. Bijzet, E. Brouwer, M.D. Posthumus, and M.H. van Rijswijk

    Imaging Human AL Amyloid in Mice Using the Fibril-Reactive Monoclonal Antibody 11-1F4, J.S. Wall, S.J. Kennel, T. Richey, J.S. Avenell, and A. Solomon

    Tandem Mass Spectrometry and Other Methods Identify Apolipoprotein E3 and Kappa 1 Light Chain in Amyloid Fibrils Extracted from a Patient with AL Amyloidosis, L. Wang, X. Yuan, B. Clark, N. Lampen, H. Deng, Y. Zhang, and R.L. Comenzo

    Mouse Models of AL Amyloidosis, J.E. Ward, D. Brenner, P. Soohoo, L. Cui, R. Liao, X. Peng, D. Sawyer, L.H. Connors, T. Christensen, C. O'Hara, M. Skinner, and D.C. Seldin

    NT-ProBNP in AL Amyloidosis: Association with Survival and Changes After Chemotherapy, A. Wechalekar, H. Lachmann, J. Gillmore, M. Offer, J. Joshi, and P. Hawkins

    Histochemical-Based Staging System for AL Amyloidosis, D.T. Weiss, C. Wooliver, and A. Solomon

    Localized AL Amyloidosis: A Distinctive Clinicopathologic Disorder, T.K. Williams, S.D. Macy, C. Wooliver, C.L. Murphy, P. Gorevic, P. Westermark, D.T. Weiss, and A. Solomon

    Evaluation of Plasma Cell Volume and Clonality in AL (primary) Amyloidosis by Analysis of Pre and Post Stem Cell Transplantation Bone Marrow Core Biopsies (BMCB), H. Xu, P. Soohoo, G. Doros, M. Skinner, and C. O'Hara

    Biochemical Characterization of Amyloid from a Rectal Amyloidoma, Z. Zaky, O. Cummings, J.J. Liepnieks, D. Rex, and M.D. Benson


    NEW DIAGNOSTICS AND EMERGING TRANSLATIONAL APPROACHES
    KEYNOTE ADDRESS: Advances in Systemic Amyloidoses: From Mechanisms to Better Care, G. Merlini

    The Diagnosis of Amyloidosis May Be Hindered by the Sampling Error, and How to Prevent It, M. Bandmann and R.P. Linke

    Serum Amyloid P Component (SAP) Concentration in Abdominal Fat Aspirates of Patients with Amyloidosis, J. Bijzet, B.P.C. Hazenberg, P.N. Hawkins, M.K. Leijsma, E. Brouwer, M.D. Posthumus, and M.H. van Rijswijk

    Biochemical Characterization of Amyloid by Endomyocardial Biopsy, J. Breall, M.D. Benson, J.J. Liepnieks, and O. Cummings

    A Peptide-Based Vaccine Elicits CTL Responses towards AL Amyloid-Related Immunoglobulin Light Chain, A. Flies and D.H. Sherr

    Diagnostic Accuracy and Prognostic Value of 123I-Labeled Serum Amyloid P Component Body Retention in Patients with Amyloidosis, B.P.C. Hazenberg, M.N. Lub-de Hooge, E. Vellenga, E.B. Haagsma, M.D. Posthumus, and P.L. Jager

    A Proteomic Approach to the Study of Systemic Amyloidoses, F. Lavatelli, D.H. Perlman, B. Spencer, T. Prokaeva, M.E. McComb, R. Théberge, L.H. Connors, V. Bellotti, D.C. Seldin, G. Merlini, M. Skinner, and C.E. Costello

    Definitive Identification of Amyloid Deposits by Mass Spectrometry, C.L. Murphy, S. Wang, T.K. Williams, D.T. Weiss, and A. Solomon

    Tissue Detection of Amyloid by an Amyloid "Chip"?, M.M. Picken, B.P.C. Hazenberg, and L. Lomas

    Detection and Characterization of Immunoglobulin Light Chain Post-Translational Modifications Using LC/MS-MS/MS, R. Théberge, Y. Jiang, M.E. McComb, T. Prokaeva, L.H. Connors, M. Skinner, D.C. Seldin, and C.E. Costello

    The Inhibitory Fc?-Receptor IIB (CD32B) Is Highly Expressed on Clonal Plasma Cells from Patients with Systemic Light-Chain (AL) Amyloidosis and Provides a Target for Monoclonal Antibody Therapy, P. Zhou, A.B. Olshen, E. Bonvini, S. Koenig, M. Fleisher, J. Young, A. Boruchov, and R.L. Comenzo


    CLINICAL SESSIONS
    DIAGNOSTIC INTERACTIVE SESSION: Report, M.M. Picken, B.P.C. Hazenberg, and L. Obici

    CLINICAL INTERACTIVE SESSION: Report, V. Sanchorawala, G. Palladini, and A. Dispenzieri

    CLINICAL TRIAL REPORTS: Clinical Trial for TTR Amyloidosis Using Diflunisal, J.L. Berk, A.B. Bisbee, J.W. Kelly, P.J. Dyck, R.H. Falk, Y. Ando, T. Coelho, S. Ikeda, G. Merlini, O.B. Suhr, M. Skinner, and the Diflunisal Investigators Consortium

    Early Harvest Followed by Melphalan-Dexamethasone and Second-Line Autologous Stem Cell Transplantation in AL, P. Russo, G. Palladini, V. Perfetti, F. Lavatelli, M. Nuvolone, L. Obici, and G. Merlini

    Treatment of AL Amyloidosis with Tandem Cycles of High Dose Melphalan and Autologous Stem Cell Transplantation: Final Analysis of a Prospective Trial , V. Sanchorawala, D.G. Wright, K. Quillen, K.T. Finn, L.M. Dember, J.L. Berk, G. Doros, C. Fisher, M. Skinner, and D.C. Seldin

    Biography

    Martha Skinner, John L. Berk, Lawreen H. Connors, David C. Seldin