Amyloidosis

ISBN-13: 9781461292920 / Angielski / Miękka / 2011 / 858 str.

George G. Glenner

Opis

From a process that from the days of Vir chow and Rokitansky, primarily stimulated the relatively narrow interest of pathologists, amyloidosis has risen full-blown as one of the most important of disease complexes. Its presence dominat: es the lesions of Alzheimer's disease, a disease affecting an estimated 2. 5 million people in the U. S. A. and thereby closely rivaling stroke as the third most common cause of death. If, as it has been de- scribed, Alzheimer's disease is the "Disease of the Century," then amy- loidosis is the Disease Complex of the Ages. It affects in one or more of its manifestations every organ of the body, and is at least as old as the afflicted Egyptian mummies of the pyramids. With an increasing percentage of older individuals amyloid of the senior population becomes increasingly more frequent. The subjects covered in this Symposium range through almost every clinical medical specialty. From an average of one paper in each of the past three Symposiums, the explosive interest in cerebral amyloidosis has led to the presentation of 12 papers on this subject in the present volume. The genetically predisposed familial amyloidotic processes, such as the polyneuropathies and familial Mediterranean fever have also stimulated ex- tensive and intriguing investigations which have revealed the striking effect of a single amino acid substitution in transforming a normal protein into. a lethal "amyloidogenic" one.

Spis treści

A. SAA, AA and AP Proteins.- Protein AA and Associated Proteins in Type-AA Amyloid Substance.- Heterogeneity of Human Amyloid Protein AA and SAA.- Vascular AA Amyloidosis is Characterized by Special Protein AA Subspecies.- The Physico-Chemical, Antigenic and Functional Heterogeneity of Human Serum Amyloid A.- Reaggregation of Bovine Amyloid A Fibril Components to ?-Pleated Sheet Fibrillar Structures.- Analysis of X-Ray Scattering by Human AA Fibrils using Secondary Structure Predictions of Human SAA1.- Characterization of Two Distinct Serum Amyloid A Gene Products Defined by Their Complementary DNAs.- Human Serum Amyloid Genes — Molecular Characterization.- Kinetics of Human Serum Amyloid A.- Ultrastructural Identification of AA-Type Amyloid Fibrils Using Polyclonal and Monoclonal Antibodies.- Human Serum Amyloid P-Component (SAP) as an Acute Phase Reactant in the Female.- B. Experimental Models.- Pathogenetic Mechanisms and Precursor Product Relationships in Murine Amyloidosis.- A Possible Effect of Oral Tolerance in Casein Induced Murine Amyloidosis?.- Isolation and Characterization of Amyloid Enhancing Factor (AEF).- Enhancement of Amyloid Degradation by Ascorbic Acid: In Vivo Evidence in a Murine Model.- Effect of Colchicine on the Acute Phase Serum Amyloid A Protein Response and Splenic Amyloid Deposition During Experimental Murine Inflammation.- Serum Amyloid A Protein (SAA) from Mink, Horse, and Man: A Comparative Study.- The Time Relationship Between Amyloid Deposition and Glycosaminoglycan Accumulation During Experimental Amyloidosis.- Kinetics of Selective Deposition of ApoSAA2 During Development of Amyloidosis in Mice.- InVivo Radioimmunodetection of Amyloid Deposits in Experimental Amyloidosis.- Prostacyclin and Thromboxane Production from Macrophages of Amyloid Resistant and Sensitive Mice.- C. In Vitro Synthesis.- Regulation of Serum Amyloid A Synthesis in Primary Mouse Hepatocyte Cultures.- Immunoelectron Microscopic Study of Liver in Experimental Murine Amyloidosis Using Anti-Mouse AA Antiserum.- Immunocytochemical Studies on the Site of Synthesis and Pathways of Amyloid Protein AA.- A Comparison of Serum Amyloid A (SAA) Synthesis With That of the Pentraxins: Serum Amyloid P (SAP) and C-Reactive Protein (CRP).- Further Studies on the Mechanism of Action of Surface Associated Proteolytic Enzymes on Lymphocytes and Monocytes Which Degrade SAA Precursor to AA-Like Products.- Down-Regulation of Kupffer Cell Ectoenzymes Precedes Deposition of Amyloid Protein A.- Specific Chemical Dissociation of Fibrillar and Non-Fibrillar Components of Amyloid Deposits.- D. Degrading Factors.- Degradation of AA-Amyloid, Clinical Experience: An Introduction.- In Vivo Degradation of Protein SAA to Protein AA and Incorporation in Amyloid Fibrils.- Isolation and Characterization of the Inhibitor of Amyloid Degrading Factor.- Amyloid-Agarose Plate Test: Ultrastructural Changes in the Fibril and Its Association with Human Neutrophil Elastase.- Decreased Esterase Activity in Serum of Patients with Reactive Systemic (AA) Amyloidosis.- Enhanced Degradation of Amyloid AA Proteins by Enzyme Activation: A Possible Model for a Therapeutic Approach.- Does Serum Degrade Amyloid Fibrils? Failure to Confirm Enzymatic Degradation of Amyloid A Fibrils as the Basis of the So-Called “Amyloid Degrading Activity” of Serum.- E. Hereditary and Familial (AF) Amyloidosis.- Statement Regarding Nomenclature for the Protein Known as Prealbumin, Which is Also (Recently) Called Transthyretin.- Family Studies of Transthyretin (Prealbumin) and Its Methionine 30 Variant in Portuguese Patients with Familial Amyloidotic Polyneuropathy.- Serum Concentrations of Prealbumin and Retinol Binding Protein in Familial Amyloid Patients From 15 Kinships.- Pre-Albumin and Retinol Binding Protein Serum Concentrations in the Brazilian Type (Portuguese) of Familial Polyneuropathy.- Clinical Investigations of Autonomic Heart Regulation and Renal Function of Familial Amyloidotic Polyneuropathy.- Identification of Amyloid Prealbumin Variant in Familial Amyloidotic Polyneuropathy of Japanese Origin: Three Patients of Different Pedigrees.- Monoclonal Antibodies to Serum Prealbumin of a Patient with Familial Amyloidotic Polyneuropathy.- Salient Structural Features of Low Molecular Weight Amyloid Fibril Proteins in Familial Amyloid Polyneuropathy of Japanese Origin.- Immunohistochemical Studies on Autopsied Organs of Familial Amyloid Polyneuropathy in Relation with Amyloid Protein.- Studies on Blood Markers and Amyloid Fibril in the Arao Focus of Familial Amyloid Polyneuropathy.- Familial Amyloidotic Polyneuropathy Type I and Type II: Characterization of Two Distinct Genetic Defects and Identification of Carriers of Each Gene.- Familial Amyloidotic Polyneuropathy Type I: Characterization of the Prealbumin Amyloid Subunit and Precursor Protein.- Immonohistochemical Studies on Hereditary Amyloidosis of the Finnish Type.- Late Onset Hereditary Amyloidosis in a Family from Texas.- Genetic Heterogeneity of Familial Amyloid Polyneuropathies of Jewish Type.- Prealbumin Nature of the Amyloid in Familal Amyloid Cardiomyopathy of Danish Origin.- Diagnosis of Familial Amyloidotic Polyneuropathy by Recombinant DNA Techniques.- Biochemical Nature of Familial Amyloidotic Polyneuropathy and a New Diagnostic Method by Radioimmunoassay.- Clinical and Pathological Studies of Familial Amyloid Polyneuropathy (FAP), with Special Reference to Nephropathy and Cardiopathy.- Cardiac Disorders and Autonomic Nervous System Involvement in Familial Amyloidosis.- Treatment of Orthostatic Hypotension in Familial Amyloid Polyneuropathy with L-Threo-3,4-Dihydroxyphenylserine.- Review of Clinical Records and Therapeutic Trials in Familial Amyloidotic Polyneuropathy (Type I) in Japan.- F. Immunoglobuin (AL) Amyloidosis.- Light Chain Variable Region Subgroups of Monoclonal Immunoglobulins in Amyloidosis AL.- Structural Studies of the Variable Region of Immunoglobulin Light-Chain-Type Amyloid Fibril Proteins.- Amyloidogenicity and Subgroups of Human Lambda Bence Jones Proteins.- Appearance of Systemic Amyloidosis in Myeloma with Amyloidogenic Bence Jones Protein.- Complete Primary Structure of an Immunoglobulin ?II-Chain Derived Amyloid Fibril Protein (HAR).- Some Structural Factors Involved in Amyloid Fibril Formation by Lambda VI Light Chain Proteins.- Polymorphism in a Kappa I Primary (AL) Amyloid Protein (Ban).- Analysis of Monoclonal Components in Systemic AL-Amyloidosis.- Structural and Immunologic Studies of a Kappa Amyloid Fibril Protein.- Molecular Heterogeneity and ?-Carboxyglutamic Acid Content of Bence-Jones Proteins: Possible Relevance to Amyloidogenicity.- Aberrant Immunoglobulin Synthesis in AL Amyloid.- G. Clinical Studies.- Primary Systemic Amyloidosis (AL): Comparison of Melphalan-Prednisone vs. Colchicine Treatment in 101 Cases.- The Life Span of Patients with Primary (AL) Amyloidosis and the Effect of Colchicine Treatment.- The Impact of Colchicine on the Amyloidosis of Familial Mediterranean Fever (FMF).- Treatment of Systemic AA Amyloidosis.- Splenic Function in Amyloidosis.- Does the Urinary Protein Pattern in AA-Amyloid Nephropathy Differ From That in Other Nephropathies?.- Classification of Amyloid Syndromes from Tissue Sections Using Antibodies Against Various Amyloid Fibril Proteins: Report of 142 Cases.- Clinical, Pathological, and Functional Findings in Amyloid Nephropathy.- Fine Needle Aspiration Biopsy of Abdominal Subcutaneous Fat Tissue for the Diagnosis and Typing of Amyloidosis.- Non-Invasive Techniques for Demonstrating Cardiac Involvement in the Acquired Forms of Systemic Amyloidosis.- Soft-Tissue Uptake of 99mTc-Disphosphonate and 99mTc-Pyrophosphate in Systemic AA and AL-Amyloidosis.- Plasma Exchange in the Treatment of Patients with Systemic Amyloidosis.- H. Aging and Amyloidosis.- Research in Aging and Amyloidosis.- Amyloid and Aging — An Hypothesis Involving So-Called Amyloid Degrading Activity (ADA).- Senile Systemic Amyloidosis.- Varied Composition and Nature of Senile Localized Amyloid: Implications for Varied Mechanisms of Pathogenesis.- A New Senile Amyloid Fibril Protein and Its Putative Precursor in Senescence Acclerated Mouse (SAM).- Amyloid Deposition in the Articular Structures of Senescence Accelerated Mouse (SAM).- Antioxidants in Experimental Amyloidosis of Young and Old Mice.- I. Cerebral Amyloidosis and Alzheimer’s Disease.- Amyloid Research as a Paradigm for Alzheimer’s Disease.- Neurobiological Probes for Specific Constituents of Senile Plaques in Aging and Alzheimer’s Disease.- Biochemical and Structural Studies of Paired Helical Filaments and Senile Plaque Amyloid in Alzheimer’s Disease.- Alzheimer Neurofibrillary Tangle and Its Relationship with Plaque Core Amyloid.- Cerebral Amyloid and Alzheimer’s Syndrome.- Prion Amyloids in Scrapie and Creutzfeldt-Jakob Disease.- Senile Cerebral Amyloid — Evidence for a Neuronal Origin of the Fibril Protein.- Isolation and Partial Characterization of Alzheimer Neurofibrillary Tangles.- Amylid Fibrils in Hereditary Cerebral Hemorrhage with Amyloidosis (HCHWA) is Related to Cystatin (Gamma Trace).- Cerebral Amyloid Angiopathy Causing Intracranial Hemorrhage, A Clinicopathological Study of 12 Cases.- The Role of Cerebral Amyloid Angiopathy in Dementia of Alzheimer’s Type (Preliminary Results).- Hereditary Central Nervous System ?- Trace Amyloid Angiopathy and Stroke in Icelandic Families.- J. Other Types of Amyloidosis.- Amyloid Associated with Calcifying Epithelial Odontogenic Tumor — A New Type of Amyloid Fibril Protein CEOT.- Immunological Homology of the Amyloid of Insulinoma and the Islet Amyloid of the Aged: Biochemical Characterization of the Insulinoma Amyloid.- Histopathology of the Cutaneous Amyloid: A Comparative Study on 144 Cases of Localized Cutaneous Amyloidosis and 20 Cases of Systemic Amyloidosis.- Histological Evidence of Amyloid Deposition in Old Thrombotic Lesions and in Longterm Bioprosthetic Cardiac Valve Implants in Man: Two Recently Observed and Possibly Related Forms of Localized Amyloidosis of the Cardiovascular System.- Contributors.