High energy resolution core-level X-ray spectroscopy for electronic and structural characterization of osmium compounds

Table of Contents......Page 6
Preface......Page 18
Outstanding Achievement Award......Page 20
Symposium Keynote Address......Page 22
Report of the Nomenclature Committee......Page 24
SECTION I: MECHANISMS OF FIBRIL FORMATION AND CELLULAR RESPONSE......Page 26
KEYNOTE ADDRESS: THE EFFECT OF MEMBRANES AND SURFACES ON THE FIBRILLATION OF AN AMYLOIDOGENIC LIGHT CHAIN: RELEVANCE TO THE ETIOLOGY OF AL AMYLOIDOSIS......Page 28
HEPARAN SULFATE PROMOTES THE REFOLDING AND OLIGOMERIZATION OF SAA1.1: A SHORT SEQUENCE IS IMPLICATED IN THIS MISFOLDING PROCESS......Page 32
MOUSE SENILE AMYLOIDOSIS: POSSIBLE HORIZONTAL TRANSMISSION IN THE MOUSE ROOM......Page 35
CHARGE DIFFERENCES OF DEPOSITS IN AL AND NALCDD......Page 38
ROLE OF THE C-TERMINAL AMINO ACIDS IN  2-MICROGLOBULIN AMYLOID FORMATION......Page 41
SERUM AMYLOID A GENERATES HIGH-DENSITY LIPOPROTEIN FROM HUMAN AORTIC SMOOTH MUSCLE CELLS......Page 44
AMYLOID LIGHT CHAINS LOCALIZE TO LYSOSOMES AND CARDIAC FIBROBLASTS SHOW REDUCED MITOCHONDRIAL FUNCTION......Page 47
CEREBROSPINAL FLUID AND PLASMA OF ALZHEIMER PATIENTS PROMOTE - AMYLOID FIBRIL FORMATION IN VITRO......Page 50
A NOVEL ASSAY FOR DETECTION OF AMYLOID INDUCED APOPTOSIS......Page 52
HUMAN AMYLOIDOGENIC IMMUNOGLOBULIN LIGHT CHAINS ALTER CARDIOMYOCYTE SIGNALING AND DISRUPT HEPARAN SULFATE PROTEOGLYCAN (HSPG) EXPRESSION......Page 55
GAGS ASSOCIATED WITH FIBRIL EXTRACTS MEDIATE FIBRILLOGENESIS IN AL AMYLOIDOSIS......Page 58
DECORIN IN AA AMYLOIDOSIS......Page 61
STRUCTURAL EVOLUTION OF IMMUNOGLOBULIN LIGHT CHAIN DURING AMYLOID FIBRIL FORMATION: A DEEP UV RESONANCE RAMAN SPECTROSCOPIC STUDY......Page 64
SECTION II: AA AND LOCALIZED AMYLOIDOSES......Page 68
KEYNOTE ADDRESS: AA AMYLOIDOGENESIS: BASIC AND TRANSLATIONAL RESEARCH......Page 70
HISTOCHEMICAL AND IMMUNOHISTOCHEMICAL CHARACTERISTICS OF AMYLOID DEPOSITS......Page 75
ULTRASTRUCTURAL CHARACTERISTICS OF AMYLOID DEPOSITS......Page 78
ODONTOGENIC FELINE AND CANINE TUMOR-ASSOCIATED AMYLOID IS FORMED FROM AMELOBLASTIN......Page 81
AMYLOIDOGENIC REGION OF ODONTOGENIC AMELOBLAST ASSOCIATED PROTEIN (ODAM)......Page 84
WHAT IS THE BEST WAY TO ASSESS PREDISPOSITION TO AA AMYLOID IN INFLAMMATORY JOINT DISEASE?......Page 87
ADVANCES IN NON-INVASIVE IMAGING OF AA AMYLOID IN MICE......Page 90
DETECTION AND IDENTIFICATION OF AMYLOID DEPOSITION ASSOCIATED WITH HUMAN UNERUPTED TOOTH FOLLICLES......Page 93
VASCULAR AA AMYLOID FORMATION MODELED IN CELL CULTURE......Page 95
AMYLOIDOSIS IN ANKYLOSING SPONDYLITIS - UNEXPECTED FINDINGS UNDERSCORING THE IMPORTANCE OF TYPING OF AMYLOID DEPOSITS......Page 98
LACK OF CORRELATION BETWEEN AMYLOID A PROTEIN LEVELS AND CLINICAL PARAMETERS IN ASPIRATED FAT TISSUE OF FAMILIAL MEDITERRANEAN FEVER PATIENTS WITH SECONDARY AMYLOIDOSIS......Page 100
N-FORMYL PEPTIDE RECEPTOR-LIKE 1 OF THE NEUTROPHILS IN THE AA-AMYLOIDOSIS TISSUES IS LESS STAINED......Page 109
AL IN THE CLINICAL SETTING OF AA: THE IMPORTANCE OF AMYLOID TYPING......Page 113
FOIE GRAS IS A DIETARY SOURCE OF AEF......Page 115
THE EFFECT OF A VARIETY OF ANTI-INFLAMMATORY DRUGS ON MURINE AMYLOIDOGENESIS......Page 118
TRANSLOCATION OF SYSTEMIC AMYLOIDOSIS BY PERIPHERAL BLOOD MONOCYTES......Page 121
FK506 INHIBITS MURINE AA AMYLOIDOSIS WITHOUT MODULATING SERUM SAA LEVELS......Page 124
LOCAL TUMOROUS AA-AMYLOID DEPOSITION IN A CASE OF HEPATIC ADENOMATOSIS: IMMUNOHISTOCHEMISTRY AND IN SITU HYBRIDIZATION EMPLOYING SAA-MRNA PROBES......Page 127
SAA4-RELATED AA AMYLOIDOSIS......Page 130
SECTION III: FAMILIAL AND TTR AMYLOIDOSES......Page 136
IN VITRO AND IN VIVO EFFECTS OF GENISTEIN ON TTR STABILIZATION AND AGGREGATION......Page 138
LABIAL SALIVARY GLAND BIOPSY FOR DIAGNOSIS OF AMYLOID POLYNEUROPATHY PORTUGUESE TYPE......Page 141
HEREDITARY GELSOLIN AMYLOIDOSIS IN AN IRANIAN FAMILY: THE FIRST REPORT FROM THE MIDDLE EAST......Page 143
A CASE OF COMPOUND HETEROZYGOSITY IN A NIGERIAN MALE WITH IDENTIFICATION OF A NEW TRANSTHYRETIN VARIANT PHE44LEU AND VAL122ILE......Page 146
SUPPRESSION OF HEPATIC TRANSTHYRETIN SYNTHESIS BY ANTISENSE OLIGONUCLEOTIDES......Page 149
TOP-DOWN MASS SPECTROMETRY ANALYSIS OF AMYLOIDOGENIC PROTEINS......Page 152
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”......Page 155
FAMILIAL AMYLOID NEPHROPATHY OF FIBRINOGEN Aα (E526V) ORIGIN. REPORT OF THE FIRST CASE FROM AUSTRIA......Page 161
LARYNGEAL AMYLOIDOSIS IN PATIENTS WITH APOLIPOPROTEIN AI L174S AND L178P......Page 164
HEREDITARY FIBRINOGEN A  -CHAIN AMYLOIDOSIS: CLINICAL FEATURES AND THE CURATIVE ROLE OF LIVER TRANSPLANTATION......Page 166
AMYLOIDOSIS IN THE TRANSGENIC MICE OF MOUSE AMYLOIDOGENIC APOLIPOPROTEIN A-II (APOA2C)......Page 168
URINARY TRANSTHYRETIN: A POSSIBLE BIOCHEMICAL MARKER IN THE EARLY STAGE OF RENAL DYSFUNCTION......Page 171
UNIQUE PHENOTYPE OF VITREOUS AMYLOIDOSIS IN DIFFERENT ENDEMIC AREAS......Page 174
COMPOSITION OF PERIPHERAL NERVE AMYLOID AFTER ORTHOTOPIC LIVER TRANSPLANT FOR FAP......Page 177
CHARACTERIZATION OF FIBRILLAR TRANSTHYRETIN IN EX VIVO CARDIAC DEPOSITS FROM CASES OF SENILE SYSTEMIC AMYLOIDOSIS......Page 179
COMPARISON OF CARDIAC AMYLOIDOSIS ASSOCIATED WITH WILD TYPE TRANSTHYRETIN AND THE ISOLEUCINE 122 VARIANT......Page 182
AL AMYLOIDOSIS IN A PATIENT WITH A T60A TTR MUTATION......Page 185
BIOCHEMICAL CHARACTERIZATION OF LEPTOMENINGEAL AMYLOID IN A VAL30GLY TRANSTHYRETIN PATIENT......Page 188
APOLIPOPROTEIN A-I (APO A-I) NATURE OF KNEE JOINT MENISCUS-ASSOCIATED AMYLOID......Page 191
AN AUTOPSY CASE OF FAMILIAL AMYLOIDOTIC POLYNEUROPATHY ATTR VAL30MET 10 YEARS AFTER LIVER TRANSPLANTATION: CHANGES IN THE AMOUNT OF AMYLOID DEPOSITS IN THE PANCREAS......Page 194
AMYLOID NEUROPATHY IN A JAPANESE DOMINO LIVER-TRANSPLANTED RECIPIENT......Page 197
THE NATURAL HISTORY OF HEREDITARY APOLIPOPROTEIN A-I AMYLOIDOSIS ACCORDING TO A LARGE SERIES OF ITALIAN LEU75PRO PATIENTS......Page 200
DOMINO LIVER TRANSPLANTATION USING THE EXPLANTED GRAFTS FROM PATIENTS WITH HEREDITARY FORMS OF AMYLOIDOSIS: THE KING\'S COLLEGE HOSPITAL EXPERIENCE......Page 203
CARDIOMYOPATHY IN FAP VAL30MET PATIENTS WITH AND WITHOUT LIVER TRANSPLANTATION......Page 205
DETECTION OF CARDIAC PRE-AMYLOID DEPOSITS OF TRANSTHYRETIN BY MALDI-TOF......Page 207
EFFECT OF NITRIC OXIDE IN TRANSTHYRETIN-RELATED AMYLOIDOSIS......Page 210
INHIBITION OF INTRACELLULAR TETRAMERIZATION INDUCES THE PROTEASOMAL DEGRADATION OF AMYLOIDOGENIC TRANSTHYRETIN VARIANTS......Page 213
STRUCTURAL ANALYSES OF FIBRINOGEN Aõ-CHAIN AMYLOID FIBRILS......Page 216
A TRANSTHYRETIN MUTATION (V32A) IN AN ISRAELI PATIENT WITH AMYLOIDOTIC NEUROPATHY LOCALIZES TO A 3D MUTATION CLUSTER......Page 218
AMYLOID DEPOSITION IN THE TONGUE OF PATIENTS WITH FAMILIAL AMYLOIDOTIC POLYNEUROPATHY......Page 221
RECENT NOVEL AND RARE MUTATIONS IN A CLINIC POPULATION OF PATIENTS WITH AMYLOIDOSIS......Page 224
PROLIFERATION OF SMOOTH MUSCLE CELLS IN THE TUNICA INTIMA OF CEREBRAL VESSELS IN FAMILIAL AMYLOID TIC POLYNEUROPATHY ATTR Y114C......Page 227
REENGINEERING TTR AMYLOID INHIBITION PROPERTIES OF DIFLUNISAL......Page 230
FAMILIAL AMYLOIDOSIS ASSOCIATED WITH A NOVEL MUTATION (D68G) IN THE LYSOZYME GENE......Page 233
EFFECTS OF LIVER TRANSPLANTATION ON HEREDITARY CEREBRAL AMYLOID ANGIOPATHY ASSOCIATED WITH ATTR Y114C......Page 236
EFFECT OF TACROLIMUS AND CYCLOSPORINE ON TRANSTHYRETIN (TTR) METABOLISM IN RATS: A LONG TERM ANALYSIS......Page 239
SECTION IV: AL AMYLOIDOSIS......Page 242
KEYNOTE ADDRESS: CAN AL AMYLOIDOSIS BE CURED?......Page 244
IDENTIFICATION OF NOVEL INHIBITORS OF LIGHT CHAIN FIBRILLOGENESIS......Page 247
PEPTIDE PHAGE DISPLAY AND EPITOPE MAPPING OF A FIBRIL-RELATED CONFORMATIONAL EPITOPE RECOGNIZED BY THE 11-1F4 MONOCLONAL ANTIBODY......Page 250
THE TRANSLOCATION T(11;14) IS FREQUENTLY DETECTED IN PATIENTS WITH AL AMYLOIDOSIS......Page 253
ALBASE: A PLATFORM ANALYSIS TOOL FOR THE STUDY OF AMYLOID LIGHT CHAIN SEQUENCE DATA......Page 256
RISK-ADAPTED MELPHALAN WITH STEM CELL TRANSPLANT (SCT) AND ADJUVANT DEXAMETHASONE (DEX) +/- THALIDOMIDE (THAL) ACHIEVES LOW TREATMENT-RELATED MORTALITY AND HIGH HEMATOLOGIC RESPONSE RATES......Page 259
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......Page 262
COMPARISON OF IMMUNOGLOBULIN LIGHT CHAINS (K1): AMYLOIDOGENIC VS. NON-AMYLOIDOGENIC PROTEINS......Page 265
CLINICOPATHOLOGICAL ANALYSIS OF THIRTY-TWO PATIENTS WITH AL AMYLOIDOSIS: FOUR-YEARS EXPERIENCE IN A SINGLE INSTITUTION......Page 268
ROLE OF LIGHT CHAIN DEAMIDATION IN THE PATHOGENESIS OF AL AMYLOIDOSIS......Page 271
THE EXTENT AND DISTRIBUTION OF AMYLOID IN 38 AUTOPSIES ON PATIENTS WITH PRIMARY (AL) AMYLOIDOSIS......Page 274
TRANSPLANTATION WITHOUT GROWTH FACTOR ENGRAFTMENT KINETICS FOLLOWING STEM CELL TRANSPLANTATION FOR PRIMARY SYSTEMIC AMYLOIDOSIS......Page 277
EXTENT OF HEMATOLOGIC RESPONSE IS IMPORTANT IN DETERMINING OUTCOME IN TRANSPLANTED PATIENTS WITH PRIMARY SYSTEMIC AMYLOIDOSIS. IMPORTANCE OF ACHIEVING A COMPLETE RESPONSE......Page 280
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)......Page 283
DETECTION AND CHARACTERIZATION OF AL LIGHT CHAIN PROTEINS FROM FIBRILS......Page 286
CHARACTERIZATION OF POST-TRANSLATIONAL MODIFICATIONS OF AN AMYLOIDOGENIC IMMUNOGLOBULIN KAPPA LIGHT CHAIN BY MASS SPECTROMETRY......Page 289
INVOLVEMENT OF LYSOSOMES IN RENAL AL-AMYLOIDOGENESIS......Page 292
THERMAL UNFOLDING OF IMMUNOGLOBULIN LIGHT CHAINS IS LINKED TO AGGREGATION: CIRCULAR DICHROISM AND LIGHT SCATTERING STUDIES......Page 295
PROGNOSTIC RELEVANCE OF SERUM N-TERMINAL PRONATRIURETIC PEPTIDE TYPE B AND PLASMA BNP IN PATIENTS WITH AL AMYLOIDOSIS IN DIALYSIS......Page 298
AL AMYLOIDOSIS IN THE ELDERLY: A REVIEW OF 330 PATIENTS......Page 300
THERAPEUTIC POTENTIAL OF NATURALLY OCCURRING FIBRIL-REACTIVE HUMAN ANTIBODIES FOR AL AMYLOIDOSIS......Page 303
PROLONGED FOLLOW-UP STUDY OF AL PATIENTS INELIGIBLE FOR STEM CELL TRANSPLANTATION TREATED WITH ORAL MELPHALAN AND DEXAMETHASONE......Page 306
LEFT VENTRICULAR SYSTOLIC FUNCTION IN PATIENTS WITH CARDIAC AL AMYLOIDOSIS......Page 308
SILENCING OF AMYLOIDOGENIC 6 LIGHT CHAIN PRODUCTION......Page 311
AMYLOID TYPING IN SURGICAL PATHOLOGY: EXPERIENCE OF A SINGLE INSTITUTION......Page 314
SOMATIC MUTATIONS CREATE POTENTIAL N-GLYCOSYLATION SITES IN THE IMMUNOGLOBULIN LIGHT CHAIN VARIABLE REGIONS IN PRIMARY AMYLOIDOSIS......Page 317
A TRIAL OF ANTIOXIDANTS TO IMPROVE CARDIAC FUNCTION IN PATIENTS WITH AL AMYLOIDOSIS AND CARDIAC INVOLVEMENT......Page 320
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......Page 323
GLYCOSYLATED AMYLOID AND NON-AMYLOID ASSOCIATED LAMBDA LIGHT CHAINS......Page 326
EXTRAMEDULLARY PRODUCTION OF AL AMYLOID PRECURSOR PROTEIN......Page 329
MESANGIAL AMYLOID FORMATION IN AN ISOLATED RAT KIDNEY PERFUSION MODEL: A NEW MODEL TO STUDY RENAL AMYLOIDOGENESIS......Page 332
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......Page 335
SEMI-QUANTITATIVE ASSESSMENT OF AMYLOID IN CONGO RED-STAINED SUBCUTANEOUS ABDOMINAL FAT TISSUE IN SYSTEMIC AMYLOIDOSIS AND DISEASE SEVERITY......Page 338
IMAGING HUMAN AL AMYLOID IN MICE USING THE FIBRIL-REACTIVE MONOCLONAL ANTIBODY 11-1F4......Page 340
TANDEM MASS SPECTROMETRY AND OTHER METHODS IDENTIFY APOLIPOPROTEIN E3 AND KAPPA 1 LIGHT CHAIN IN AMYLOID FIBRILS EXTRACTED FROM A PATIENT WITH AL AMYLOIDOSIS......Page 343
MOUSE MODELS OF AL AMYLOIDOSIS......Page 346
NT-PROBNP IN AL AMYLOIDOSIS: ASSOCIATION WITH SURVIVAL AND CHANGES AFTER CHEMOTHERAPY......Page 349
HISTOCHEMICAL-BASED STAGING SYSTEM FOR AL AMYLOIDOSIS......Page 352
LOCALIZED AL AMYLOIDOSIS: A DISTINCTIVE CLINICOPATHOLOGIC DISORDER......Page 355
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)......Page 358
BIOCHEMICAL CHARACTERIZATION OF AMYLOID FROM A RECTAL AMYLOIDOMA......Page 361
SECTION V: NEW DIAGNOSTICS AND EMERGING TRANSLATIONAL APPROACHES......Page 364
KEYNOTE ADDRESS: ADVANCES IN SYSTEMIC AMYLOIDOSES: FROM MECHANISMS TO BETTER CARE......Page 366
THE DIAGNOSIS OF AMYLOIDOSIS MAY BE HINDERED BY THE SAMPLING ERROR, AND HOW TO PREVENT IT......Page 372
SERUM AMYLOID P COMPONENT (SAP) CONCENTRATION IN ABDOMINAL FAT ASPIRATES OF PATIENTS WITH AMYLOIDOSIS......Page 375
BIOCHEMICAL CHARACTERIZATION OF AMYLOID BY ENDOMYOCARDIAL BIOPSY......Page 377
A PEPTIDE-BASED VACCINE ELICITS CTL RESPONSES TOWARDS AL AMYLOID-RELATED IMMUNOGLOBULIN LIGHT CHAIN......Page 380
DIAGNOSTIC ACCURACY AND PROGNOSTIC VALUE OF 123I-LABELED SERUM AMYLOID P COMPONENT BODY RETENTION IN PATIENTS WITH AMYLOIDOSIS......Page 383
A PROTEOMIC APPROACH TO THE STUDY OF SYSTEMIC AMYLOIDOSES......Page 385
DEFINITIVE IDENTIFICATION OF AMYLOID DEPOSITS BY MASS SPECTROMETRY......Page 388
TISSUE DETECTION OF AMYLOID BY AN AMYLOID \"CHIP\"?......Page 390
DETECTION AND CHARACTERIZATION OF IMMUNOGLOBULIN LIGHT CHAIN POST-TRANSLATIONAL MODIFICATIONS USING LC/MS-MS/MS......Page 393
THE INHIBITORY Fcy-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......Page 396
SECTION VI: CLINICAL SESSIONS......Page 400
REPORT FROM THE DIAGNOSTIC INTERACTIVE SESSION......Page 402
REPORT FROM THE CLINICAL INTERACTIVE SESSION......Page 408
CLINICAL TRIAL FOR TTR AMYLOIDOSIS USING DIFLUNISAL......Page 412
EARLY HARVEST FOLLOWED BY MELPHALAN-DEXAMETHASONE AND SECOND-LINE AUTOLOGOUS STEM CELL TRANSPLANTATION IN AL......Page 415
TREATMENT OF AL AMYLOIDOSIS WITH TANDEM CYCLES OF HIGH DOSE MELPHALAN AND AUTOLOGOUS STEM CELL TRANSPLANTATION: FINAL ANALYSIS OF A PROSPECTIVE TRIAL......Page 418
Author Index......Page 422
Subject Index......Page 428