附註:Includes bibliographical references and indexes.
Preface -- Chapter I: Clinical Features and Genetics of MODY -- Natural History, Genetics and Pathogenesis of HNF-4Ü/MODY1 -- Phenotype-Genotype Relationships in Maturity-Onset Diabetes of the Young -- Medical Genetics of MODYs -- Chapter II: Islet Function and MODYs -- Coupling of SUR1 and K[sub(IR)]6.2 Specify the Properties of [beta]-Cell K[sub(ATP)] Channels -- Metabolic Mechanisms and Diabetes Candidate Genes: Insights Gained from Genetic Engineering with Recombinant Adenoviruses -- Isolation, Characterization, and Mapping of Human Islet Transcription Factors.
Chapter III: Pathophysiology of MODYs -- The Glucokinase System and the Regulation of Blood Sugar -- Molecular Modeling of Human Glucokinase. -- Cell-Specific Roles for Glucokinase in Glucose Homeostasis and the Pathogenesis of Hyperglycemia in MODY2 as Determined by Studies in Mice -- Control of Glucose Phosphorylation/Dephosphorylation in the Liver -- Hepatic Glycogen Metabolism in Type 1 Diabetic and Glucokinase-Deficient Subjects -- Chapter IV: Islet Function and Nuclear Factors -- Role of PDX-1 in Pancreatic Development -- Molecular Mechanisms of PDX-1 Regulation.
Role of Homeodomain Transcription Factor IPF-1 in the Pathogenesis of Diabetes mellitus -- IRS-2 Is a Common Element in Peripheral Insulin Signaling and [beta]-Cell Function -- Chapter V: Hepatic Nuclear Factors -- Regulation of Visceral Endoderm Differentiation and Gene Expression by the MODY1 Transcription Factor HNF-4[alpha] -- HNF4 as a Liver Gene Transcription Factor -- Role of the Hepatocyte Nuclear Factor Network in Maturity-Onset Diabetes of the Young (MODY) -- Author Index -- Subject Index -- A -- B -- C -- D -- F -- G -- H -- I -- K -- L -- M -- N -- P -- S -- T -- U.
摘要:A timely collection of research papers by 18 leading scientists in the area of biochemical genetics of diabetes mellitus, these contributions focus on a well defined class of monogenic forms of the disease (i.e. MODYs 1-5) all caused by specific mutations or deletion of genes that control pancreatic beta-cell functions: HNF-4alpha (1), glucokinase (2), HNF-1alpha (3), IDX-1 (4) and HNF-1beta (5). This volume offers a historical perspective of MODYs, and it also contains contributions which deal with the fundamental questions of pancreas and liver development, critical for the understanding of the syndrome complex discussed. Attempts have been made to highlight results and concepts that may be relevant for understanding type-II diabetes in general, exemplified by contributions that connect the field of pancreatic islet cell biology and pathology with the newest findings in the area of insulin action, e.g. the role of the insulin receptor and insulin receptor substrates (IRS 1-3). Covering a broad and novel approach on the subject of MODYs, this book looks into the future of molecular genetic and biochemical research in this area. It is recommended reading for basic research scientists in the area of diabetes mellitus, academically oriented endocrinologists and clinical researchers.
