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Chemical structure --- Organic chemistry --- organische chemie --- protein-engineering --- eiwitten
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Hereditary tyrosinemia type 1 (HT1), the most severe inborn error of the tyrosine degradation pathway, is due to a deficiency in fumarylacetoacetate hydrolase (FAH). The worldwide frequency of HT1 is one per 100,000 births, but some regions have a significantly higher incidence (1:1,800). The FAH defect results in the accumulation of toxic metabolites, mainly in the liver. If left untreated, HT1 is usually fatal before the age of two. HT1 patients develop several chronic complications including cirrhosis with a high risk of hepatocellular carcinoma (HCC) and neuropsychological impairment. Treatment comprises an inhibitor of the pathway, Nitisinone, a strict dietary treatment or liver transplantation. Early treatment is important to avoid HCC. The book includes the latest developments on the molecular basis of HT1, its pathology, screening and diagnosis and management of the disease written by leading scientists, geneticists, hepatologists and clinicians in the field.
Chemical structure --- General biochemistry --- protein-engineering --- biochemie --- eiwitten --- moleculaire biologie
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The aim of the Protein Reviews is to serve as a publication vehicle for review articles that focus on crucial current vigorous aspects of protein structure, function, evolution and genetics. Volume 17 of Protein Reviews is the beginning of a new publication format. The volumes will appear online before they are published in a printed book. Articles will be selected according to their importance to the understanding of biological systems, their relevance to the unravelling of issues associated with health and disease or their impact on scientific or technological advances and developments. The chapters in this volume are authored by experts in the field. They deal with aspects of structure and biological activity of selected proteins. Specific chapters deal with the aggregation of FET proteins (FUS, EWSR1, TAF15) as a pathological change in amyotrophic lateral sclerosis, structural changes fundamental to gating of the cystic fibrosis transmembrane conductance regulator anion channel pore, the dual roles for epithelial splicing regulatory proteins 1 (ESRP1) and 2 (ESRP2) in cancer progression, controlling autolysis during flagella insertion in Gram-negative bacteria, the regulation of skeletal muscle myoblast differentiation and the proliferation by pannexins, hyaluronidase and chondroitinase, factors that control mitotic spindle elongation, how secreted phospholipase A2 type IIA (sPLA2-IIA) activates integrins in an allosteric manner, the simple and unique allosteric machinery of Thermus caldophilus lactate dehydrogenase, and the reduction of chemically stable multibonds: Nitrogenase-like biosynthesis of tetrapyrroles. This volume is intended for research scientists, clinicians, physicians, and graduate students in fields of biochemistry, cell biology, molecular biology microbiology, immunology and genetics. .
Chemical structure --- General biochemistry --- moleculaire structuur --- biochemie --- eiwitten --- moleculaire biologie
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This volume of the established Subcellular Biochemistry series presents 20 chapters dealing with a broad range of interesting protein complexes. It will enable researchers to readily appreciate the major contribution from both X-ray crystallography and cryo-electron microscopy in this field of study. The biological significance of these structural studies is emphasised throughout the book. The diversity of the material included here indicates the breadth of this field and the tremendous progress that has been made in recent years. The book is directed primarily to advanced students and researchers in structural biology, and others in the biochemical sciences. It will be supplemented by other related books within the Subcellular Biochemistry series. One of the Editors (JM-W) is actively involved in structural biology and the other (JRH), as a retired academic and the Series Editor of Subcellular Biochemistry, has long experience at editing multi-author books.
Chemical structure --- General biochemistry --- protein-engineering --- biochemie --- eiwitten --- moleculaire biologie
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The Subcellular Biochemistry series has recently embarked upon an almost encyclopaedic coverage of topics relating to the structure and function of macromolecular complexes (Volumes 82, 83 and 87). The present multi-author text covers numerous aspects of current research into molecular virology, with emphasis upon viral protein and nucleoprotein structure and function. Structural data from cryo-electron microscopy and X-ray crystallography is displayed throughout the book. The 17 chapters in the book cover diverse interesting topics, all currently under investigation, contributed by authors who are active actively involved in present-day research. Whilst structural aspects predominate, there is much consideration of the structure-function relationship. In addition, the book correlates with and extends from Volume 68 of the series “Structure and Physics of Viruses: An Integrated Textbook”. This book is directed primarily at professionals that work in the broad field of Structural Biology and will be of particular interest to Structural Virologists. The editors, David Bhella and Robin Harris, have much experience in virology and protein structure, respectively. Dr Bhella is Director of the Scottish Macromolecular Imaging Centre. Professor Robin Harris is the long-standing Series Editor of the Subcellular Biochemistry series. He has edited and contributed to several books in the series.
Chemical structure --- General biochemistry --- protein-engineering --- biochemie --- eiwitten --- moleculaire biologie
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Mucoproteins --- Mucopolysaccharides --- Mucopolysaccharides. --- Mucoproteins. --- biochemie --- biochemistry --- biofysica --- biophysics --- nederland --- netherlands --- Proteins and Enzymes --- Eiwitten en enzymen
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Chemical structure --- Food science and technology --- voedingschemie --- protein-engineering --- voedingsleer --- eiwitten
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Chemical structure --- Biomathematics. Biometry. Biostatistics --- protein-engineering --- bio-informatica --- eiwitten --- biometrie --- moleculaire biologie
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Chemical structure --- Histology. Cytology --- Molecular biology --- protein-engineering --- cytologie --- histologie --- eiwitten --- moleculaire biologie
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Chemical structure --- Histology. Cytology --- General biochemistry --- Molecular biology --- protein-engineering --- biochemie --- cytologie --- histologie --- eiwitten --- moleculaire biologie
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