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Abnormal proteins are known to be associated with various pathologies. Most notably, these include amyloidoses, monoclonal protein deposits associated with plasma cell dyscrasia/multiple myeloma, cryoglobulins and various related organized and non-organized deposits. Amyloid and Related Disorders presents an overview of the most recent developments in this area including clinical presentation, etiology, pathogenesis, and differential diagnosis. The rationale for various therapies, including transplantation, is discussed and tissue diagnosis (its pitfalls and strategies for avoiding them) and laboratory support are included. The involvement of all major organ systems including renal/genitourinary, cardiac, gastrointestinal, pulmonary, peripheral nerve/central nervous system, soft tissue and bone marrow pathology is covered. This approach provides a unifying concept of these pathologic processes, which have systemic involvement, and which have, hitherto, not been universally appreciated. Awareness of these diseases among a wider audience of pathologists may increase the rate of their diagnosis as well as that of earlier diagnosis. This volume will be invaluable to specialized and general pathologists as well as cytopathologists; other medical professionals may also benefit from this concise update on the systemic amyloidoses.
Amyloidosis. --- Multiprotein Complexes --- Proteostasis Deficiencies --- Proteins --- Amino Acids, Peptides, and Proteins --- Macromolecular Substances --- Metabolic Diseases --- Nutritional and Metabolic Diseases --- Chemicals and Drugs --- Diseases --- Amyloidosis --- Amyloid --- Medicine --- Health & Biological Sciences --- Pathology --- Metabolism --- Disorders. --- Protein metabolism disorders --- Amyloid degeneration --- Medicine. --- Internal medicine. --- Nephrology. --- Pathology. --- Medicine & Public Health. --- Internal Medicine. --- Lymphoproliferative disorders --- Disorders --- Medicine, Internal --- Internal medicine --- Kidneys --- Disease (Pathology) --- Medical sciences --- Medicine, Preventive
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An up-to-date reference on this fascinating set of complex disorders, this book features the most comprehensive strategies for diagnosing, classifying, imaging, treating, and managing amyloidosis in multiple organ systems. Beneficial to the spectrum of practitioners from residents to sub-specialists, this book is a succinct authoritative text written by leaders in the field. The authors provide instruction on all forms of amyloidosis - including primary amyloidosis (AL), secondary amyloidosis (AA), and familial amyloidosis. With essential treatment algorithms, Amyloidosis: Diagnosis and Treatment is the gold-standard for all hematologists, oncologists, and internists caring for patients with this disease.
Amyloid. --- Amyloidosis. --- Amyloidosis --- Natural Science Disciplines --- Analytical, Diagnostic and Therapeutic Techniques and Equipment --- Proteins --- Multiprotein Complexes --- Proteostasis Deficiencies --- Amino Acids, Peptides, and Proteins --- Macromolecular Substances --- Metabolic Diseases --- Disciplines and Occupations --- Amyloid --- Diagnosis --- Chemistry --- Nutritional and Metabolic Diseases --- Chemicals and Drugs --- Diseases --- Medicine --- Health & Biological Sciences --- Pathology --- Treatment --- Diagnosis. --- Treatment. --- Amyloid degeneration --- Medicine. --- Internal medicine. --- Hematology. --- Medicine & Public Health. --- Internal Medicine. --- Lymphoproliferative disorders --- Metabolism --- Disorders --- Haematology --- Internal medicine --- Blood --- Medicine, Internal
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There is a strong interest in transthyretin (TTR) in connection with protein evolution, medical and clinical research. Thus, this is an exciting time for experts in TTR research to come together to write a monograph covering both the basic and the clinical research into TTR. Transthyretin is a protein found in human blood and cerebrospinal fluid. It is directly involved in the transport of thyroid hormones, and indirectly in that of retinol. These hormones are essential for normal growth and development, particularly that of the brain. Spontaneous and inherited diseases affecting transthyretin result in amyloidosis. More than 80 point mutations in transthyretin lead to a variety of illnesses. Liver transplants are the most common treatment, although much research is also being carried out in drug therapies. The evolution of transthyretin has been extensively investigated – from humans to bacteria, invertebrate animals and plants. The structure of the protein has not changed, but its function has changed significantly. This is a most exciting example for the study of the evolution of protein structure-function relationships. This monograph will bring the reader up to date on the latest developments and discoveries.
Carrier proteins. --- Cerebrospinal fluid proteins. --- Thyroid hormones. --- Thyroxine. --- Cerebrospinal fluid proteins --- Thyroxine --- Carrier proteins --- Thyroid hormones --- Evolution, Molecular --- Multiprotein Complexes --- Prealbumin --- Amyloidosis --- Macromolecular Substances --- Biological Evolution --- Serum Albumin --- Proteostasis Deficiencies --- Chemicals and Drugs --- Metabolic Diseases --- Biological Processes --- Genetic Processes --- Blood Proteins --- Albumins --- Nutritional and Metabolic Diseases --- Proteins --- Biological Phenomena --- Genetic Phenomena --- Amino Acids, Peptides, and Proteins --- Phenomena and Processes --- Diseases --- Physiology --- Animal Biochemistry --- Human Anatomy & Physiology --- Health & Biological Sciences --- Blood proteins --- Liver --- Metabolism. --- Secretions. --- Plasma proteins --- Serum proteins --- Medicine. --- Human physiology. --- Endocrinology. --- Proteomics. --- Biomedicine. --- Human Physiology. --- Abdomen --- Biliary tract --- Blood plasma --- Serum --- Human biology --- Medical sciences --- Human body --- Molecular biology --- Internal medicine --- Hormones --- Endocrinology .
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Life is produced by the interplay of water and biomolecules. This book deals with the physicochemical aspects of such life phenomena produced by water and biomolecules, and addresses topics including "Protein Dynamics and Functions", "Protein and DNA Folding", and "Protein Amyloidosis". All sections have been written by internationally recognized front-line researchers. The idea for this book was born at the 5th International Symposium "Water and Biomolecules", held in Nara city, Japan, in 2008.
Amyloidosis --Genetics. --- Nervous system --Degeneration --Genetics. --- Protein folding. --- Water chemistry. --- Protein folding --- Amyloidosis --- Nervous system --- Water chemistry --- Chemistry --- Protein Folding --- Genetics --- Water --- Neurodegenerative Diseases --- Nervous System Diseases --- Oxides --- Biology --- Biochemical Processes --- Proteostasis Deficiencies --- Natural Science Disciplines --- Biophysical Processes --- Hydroxides --- Physical Processes --- Biochemical Phenomena --- Inorganic Chemicals --- Biophysical Phenomena --- Metabolic Diseases --- Biological Science Disciplines --- Oxygen Compounds --- Diseases --- Chemical Processes --- Disciplines and Occupations --- Nutritional and Metabolic Diseases --- Chemicals and Drugs --- Chemical Phenomena --- Physical Phenomena --- Phenomena and Processes --- Animal Biochemistry --- Biology - General --- Human Anatomy & Physiology --- Health & Biological Sciences --- Degeneration --- Biomolecules. --- Water. --- Biological molecules --- Life sciences. --- Proteomics. --- Life Sciences. --- Life Sciences, general. --- Hydrology --- Molecules --- Molecular biology --- Proteins --- Biosciences --- Sciences, Life --- Science
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This volume of the Subcellular Biochemistry series is devoted to the aggregation and fibrillogenesis of the amyloid-forming peptides and proteins, including emphasis on oligomer formation as well as fibril formation. The diverse topics included here are presented within 22 chapters, covering many of the relevant topics from both a basic science and clinical perspective. The authorship of these chapters is thoroughly international, with authoritative contributions from active researchers. Fundamental structural and cellular studies using many different technical approaches are presented, leading through to clinical and therapeutic aspects of the amyloid diseases, considered from both pharmaceutical and natural product view points. It is hoped that this book will be of use to both biomedical scientists and clinicians wishing to keep abreast of this rapidly advancing field, of direct importance to the understanding of cerebral neurodegenerative disease and systemic amyloid disease.
Amyloid. --- Amyloidosis -- Diagnosis. --- Amyloidosis -- Treatment. --- Amyloidosis. --- Amyloid beta-protein --- Aggregation (Chemistry) --- Amyloidosis --- Proteins --- Proteostasis Deficiencies --- Biophysical Processes --- Multiprotein Complexes --- Pathological Conditions, Anatomical --- Biochemical Processes --- Cell Movement --- Physical Processes --- Metabolic Diseases --- Cell Physiological Processes --- Amino Acids, Peptides, and Proteins --- Macromolecular Substances --- Biochemical Phenomena --- Chemical Processes --- Biophysical Phenomena --- Pathological Conditions, Signs and Symptoms --- Nutritional and Metabolic Diseases --- Diseases --- Chemical Phenomena --- Physical Phenomena --- Chemicals and Drugs --- Cell Physiological Phenomena --- Phenomena and Processes --- Protein Folding --- Plaque, Amyloid --- Amyloid --- Cell Aggregation --- Human Anatomy & Physiology --- Health & Biological Sciences --- Animal Biochemistry --- Metabolism --- Disorders. --- Protein metabolism disorders --- Amyloid degeneration --- Medicine. --- Entomology. --- Biomedicine. --- Biomedicine general. --- Medicine/Public Health, general. --- Lymphoproliferative disorders --- Disorders --- Insects --- Zoology --- Clinical sciences --- Medical profession --- Human biology --- Life sciences --- Medical sciences --- Pathology --- Physicians --- Health Workforce --- Biomedicine, general. --- Medicine --- Biology --- Invertebrates. --- Medical sciences. --- Biomedical Research. --- Invertebrate Zoology. --- Health Sciences. --- Research. --- Invertebrata --- Animals --- Biological research --- Biomedical research --- Basic medical sciences --- Basic sciences, Medical --- Biomedical sciences --- Health sciences --- Preclinical sciences --- Sciences, Medical
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Reviews the molecular events initiated by unfolded or misfolded proteins leading to conformational human diseases, with special emphasis on the macromolecular homo- and heteroassociations of the malfolded proteins into characteristic ultrastructures found primarily in Parkinson's and Alzheimer's diseases.
Nervous system -- Degeneration -- Pathophysiology. --- Protein folding. --- Proteins -- Denaturation. --- Protein Folding --- Proteostasis Deficiencies --- Neurodegenerative Diseases --- Metabolic Diseases --- Biophysical Processes --- Biochemical Processes --- Nervous System Diseases --- Diseases --- Chemical Processes --- Nutritional and Metabolic Diseases --- Physical Processes --- Biophysical Phenomena --- Biochemical Phenomena --- Physical Phenomena --- Chemical Phenomena --- Phenomena and Processes --- Neurology --- Animal Biochemistry --- Medicine --- Human Anatomy & Physiology --- Health & Biological Sciences --- Nervous system --- Degeneration --- Pathophysiology. --- Folding of proteins --- Proteins --- Folding --- Medicine. --- Molecular biology. --- Neurosciences. --- Medical biochemistry. --- Cell biology. --- Neurobiology. --- Biomedicine. --- Biomedicine general. --- Molecular Medicine. --- Cell Biology. --- Medical Biochemistry. --- Organs (Anatomy) --- Neurosciences --- Conformation --- Cytology. --- Biochemistry. --- Biological chemistry --- Chemical composition of organisms --- Organisms --- Physiological chemistry --- Biology --- Chemistry --- Medical sciences --- Cell biology --- Cellular biology --- Cells --- Cytologists --- Neural sciences --- Neurological sciences --- Neuroscience --- Clinical sciences --- Medical profession --- Human biology --- Life sciences --- Pathology --- Physicians --- Composition --- Health Workforce --- Biomedicine, general. --- Medical biochemistry --- Pathobiochemistry --- Pathological biochemistry --- Biochemistry --- Molecular biochemistry --- Molecular biophysics --- Biophysics --- Biomolecules --- Systems biology
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The health of the proteome depends upon protein quality control to regulate the proper synthesis, folding., translocation, and clearance of proteins. The cell is challenged constantyl by environmental and physiological stress, aging, and the chronic expressions of disease associated misfolded proteins. Substantial evidence supports the hypothesis that the expression of damaged proteins initiates a cascade of molecular events that leads to Alzheimer's disease, Parkinson's disease, amyotrophic lateral sclerosis, Huntington's disease, and other diseases of protein conformation.
Nervous system -- Degeneration -- Etiology. --- Nervous system -- Degeneration -- Pathophysiology. --- Nervous system -- Degeneration -- Treatment. --- Neurodegenerative diseases -- Therapy. --- Protein folding. --- Proteins -- Conformation. --- Nervous system --- Metabolic Phenomena --- Nervous System Diseases --- Proteins --- Medicine --- Molecular Conformation --- Metabolic Diseases --- Amino Acids, Peptides, and Proteins --- Nutritional and Metabolic Diseases --- Phenomena and Processes --- Molecular Structure --- Health Occupations --- Diseases --- Disciplines and Occupations --- Chemicals and Drugs --- Biochemical Phenomena --- Chemical Phenomena --- Neurodegenerative Diseases --- Protein Conformation --- Pathology --- Proteome --- Metabolism --- Proteostasis Deficiencies --- Health & Biological Sciences --- Neurology --- Degeneration --- Degeneration. --- Degeneration, Nerve --- Nerve degeneration --- Nervous system degeneration --- Neurodegenerative disease --- Neurodegenerative diseases --- Neurodegenerative disorders --- Neuron degeneration --- Nissl degeneration --- Retrograde degeneration --- Wallerian degeneration --- Degeneration and regeneration --- Medicine. --- Molecular biology. --- Neurosciences. --- Neurochemistry. --- Biomedicine. --- Molecular Medicine. --- Degeneration (Pathology) --- Clinical sciences --- Medical profession --- Human biology --- Life sciences --- Medical sciences --- Physicians --- Biochemistry --- Neurosciences --- Neural sciences --- Neurological sciences --- Neuroscience --- Health Workforce --- Molecular biochemistry --- Molecular biophysics --- Biophysics --- Biomolecules --- Systems biology
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This book, written by members of the European network PROTEOSTASIS, provides an up-to-date review of the research regarding protein homeostasis in health and disease. With new discoveries contributing to the increasing complexity of this topic, the book offers a detailed overview of the pathways regulating protein homeostasis, including autophagy and the ubiquitin protein family. Following a basic introduction, it explains how defects in protein homeostasis contribute to numerous pathologies, including cancer, neurodegeneration, inflammation and a number of rare diseases. In addition, it discusses, the role of protein homeostasis in cellular development and physiology. Highlighting the latest research in the field of protein homeostasis and its implications for various clinically relevant diseases, the book appeals to researchers and clinicians, while also offering a reference guide for scholars who are new to the field.
Proteomics. --- Molecular biology --- Proteins --- Proteasome Endopeptidase Complex. --- Proteostasis. --- Ubiquitin-Protein Ligases. --- E3 Ligase --- E3 Ubiquitin Ligase --- Ubiquitin Ligase E3 --- Ubiquitin-Protein Ligase E3 --- Ubiquitin-Protein Ligase --- Ligase E3, Ubiquitin --- Ligase E3, Ubiquitin-Protein --- Ligase, E3 --- Ligase, E3 Ubiquitin --- Ligase, Ubiquitin-Protein --- Ligases, Ubiquitin-Protein --- Ubiquitin Ligase, E3 --- Ubiquitin Protein Ligase --- Ubiquitin Protein Ligase E3 --- Ubiquitin Protein Ligases --- Protein Homeostasis --- Homeostases, Protein --- Homeostasis, Protein --- Protein Homeostases --- Proteostases --- Protein Folding --- Proteostasis Deficiencies --- 20S Proteasome --- Ingensin --- Macropain --- Macroxyproteinase --- Multicatalytic Endopeptidase Complex --- Multicatalytic Proteinase --- Prosome --- Proteasome --- Complex, Multicatalytic Endopeptidase --- Complex, Proteasome Endopeptidase --- Endopeptidase Complex, Multicatalytic --- Endopeptidase Complex, Proteasome --- Proteasome, 20S --- Proteinase, Multicatalytic --- Cell biology. --- Neurosciences. --- Immunology. --- Medicine. --- Cancer research. --- Cell Biology. --- Biomedicine, general. --- Cancer Research. --- Cancer research --- Health Workforce --- Immunobiology --- Life sciences --- Serology --- Neural sciences --- Neurological sciences --- Neuroscience --- Medical sciences --- Nervous system --- Cell biology --- Cellular biology --- Biology --- Cells --- Homeòstasi --- Proteïnes --- Immunologia --- Malalties --- Malaltia --- Medicina --- Discapacitat física --- Malalties cardiovasculars --- Malalties constitucionals --- Malalties de l'aparell digestiu --- Malalties de l'aparell genital --- Malalties de l'aparell respiratori --- Malalties de la pell --- Malalties del sistema nerviós --- Malalties dels animals --- Malalties hematològiques --- Malalties hereditàries --- Malalties de l'aparell locomotor --- Malalties d'origen nutricional --- Malalties iatrogèniques --- Malalties immunitàries --- Malalties otorrinolaringològiques --- Malalties rares --- Oftalmopaties --- Serveis d'urgències mèdiques --- Síndromes --- Trastorns de la nutrició --- Trastorns del metabolisme --- Epidemiologia --- Biologia --- Complements (Immunitat) --- Immunofarmacologia --- Immunologia clínica --- Immunologia de la trasplantació --- Neuroimmunologia --- Resposta immunitària --- Sistema immunitari --- Biomolècules --- Albúmines --- Apoproteïnes --- Conformació de proteïnes --- Enzims --- Factors de transcripció --- Glicoproteïnes --- Lipoproteïnes --- Nucleoproteïnes --- Pèptids --- Prions --- Protamines --- Proteïnes citosquelètiques --- Proteïnes de membrana --- Proteïnes de la sang --- Proteïnes recombinants --- Proteïnes supressores de tumors --- Receptors cel·lulars --- Ubiqüitina --- Xaperones moleculars --- Proteòmica --- Autorregulació (Fisiologia) --- Humors corporals --- Sistemes de control biològic --- Equilibri àcid-base --- Osmoregulació --- Cytology. --- Medicine—Research. --- Biology—Research. --- Cancer. --- Neuroscience. --- Biomedical Research. --- Cancer Biology. --- Cancers --- Carcinoma --- Malignancy (Cancer) --- Malignant tumors --- Tumors
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