Inborn Disorders of Sphingolipid Metabolism


Book Description

Inborn Disorders of Sphingolipid Metabolism is a collection of papers presented at the Third International Symposium on the Cerebral Sphingolipidoses and Allied Diseases, held at the Isaac Albert Research Institute of the Jewish Chronic Disease Hospital and at the State University of New York, Downstate Medical Center, on October 25 and 26, 1965. This book is organized into three parts encompassing 35 chapters. Part I deals first with electron microscopic, histochemical, and morphological investigations of certain sphingolipid metabolism disorders. This part also examines several case reports on the features and symptoms of spongy degeneration of the central nervous system, familial leukodystrophy, adrenal insufficiency, and cutaneous melanosis. Part II surveys the metabolism, biosynthesis, and structure of gangliosides and sialic acids. This part also considers the nature of the lipophilic portions of the brain gangliosides. This part particularly looks into the features and clinical manifestation of Tay-Sachs disease. The third part covers the genetic and clinical aspects of the Tay-Sachs disease. This part also evaluates the genetics of the Hurler-Hunter syndrome, Batten-Spielmeyer-Vogt disease, and lipogranulomatosis syndrome. This book is of value to biochemists, histochemists, geneticists, and researchers in the allied fields of lipidosis.




Sphingolipids: Basic Science and Drug Development


Book Description

Sphingolipids are lipid components of the plasma membrane in eukaryotic cells. They have an important function in signaling mechanisms in the cell. This book on sphingolipids provides insights into the basics of sphingolipid biology and drug development, with a particular emphasis on the sphingolipid derivative ceramide. In the first part basic functions of sphingolipids are described, as well as the genetics of important enzymes, sphingolipid metabolism and synthesis. The second part of this first volume focuses on drug development and pharmacology. The book is intended for scientists in pharmacology, biochemistry and cell biology with a focus on biomedical research as well as for clinicians working in pharmacology, oncology, cardiology, neurology and infectious disease. Together with Volume 216 by the same editors, the collection represents a unique, comprehensive work on sphingolipids, providing information on both sphingolipids’ basic biology (including synthesis, metabolism and cell biology) and their important function in a (patho-)physiological context.




Sphingolipids as Signaling and Regulatory Molecules


Book Description

This book attempts to analyze the latest discoveries in sphingolipid biology and how the alteration of their metabolism leads to altered signaling events and to the development of pathobiological disorders, such as cancer, cardiovascular diseases, asthma, diabetes, inflammation and infectious diseases.




Lipid Transfer in Lipoprotein Metabolism and Cardiovascular Disease


Book Description

This book provides an up-to-date review of the fundamentals of lipid metabolism and its role in cardiovascular diseases. Focusing on lipid transfer proteins in the circulation and cells, the role of important lipid transporters, the effect of recently discovered lipid binding proteins, and the link between lipid metabolism disorders and cardiovascular diseases, it covers phospholipid transfer protein, cholesteryl ester transfer protein, lipopolysaccharide binding protein, microsomal triglyceride transfer protein, ABC binding cassette members, and more. The book offers graduate students and researchers a coherent overview of lipid transfer and transport, as well as the limitations of current research in the field, and promotes further studies on cardiovascular diseases, as well as pharmaceutical research on drug discovery based on lipid transfer, transport, and binding.




Sphingolipids in Disease


Book Description

Sphingolipids are lipid components of the plasma membrane of eukaryotic cells with an important function in signaling mechanisms in the cell. This book provides insight into the physiological and pathophysiological role of sphingolipids and in particular its derivative ceramide. The function of Sphingolipids in cell signaling with regard to infectious and lung diseases, cancer, cardiovascular diseases and neuropsychiatric disorders are described and treated in distinct parts. Together with Volume 215 from the same Editors, the collection represents a unique, comprehensive work on Sphingolipids, providing information on both: Sphingolipid basic biology as well as its important function in a (patho)physiological context. The book is written for scientists in pharmacology, biochemistry and cell biology with a focus on biomedical research as well as for clinicians in pharmacology, oncology, cardiology, neurology and infectious disease. ​




Sphingolipid Biology


Book Description

Sphingolipids are fundamental to the structures of cell membranes, lipoproteins, and the stratum cornea of the skin. Many complex sphingolipids, as well as simpler sphingoid bases and derivatives, are highly bioactive as extra- and intracellular regulators of growth, differentiation, migration, survival, senescence, and numerous cellular responses to stress. This book reviews exciting new developments in sphingolipid biology/sphingolipidology that challenge our understanding of how multicellular organisms grow, develop, function, age, and die.




Bioactive Ceramides in Health and Disease


Book Description

This book is about the various roles of bioactive ceramides and other sphingolipids in cellular biology. The enigmatic biophysical and biochemical properties of ceramides and their propensity to influence membranes whether as rafts or protein-permeable channels are heavily discussed. Metabolism of ceramides and their metabolites is also focused with ceramide synthase family of proteins being a target of extensive review. Ceramide 1-phosphate and other sphingolipids are also presented in cellular physiology and pathophysiology. Prokaryotic origins of mitochondria at the level of membranes and the occurrence of apoptosis in bacteria are presented. Many aspects of ceramide and sphingolipid biology are addressed in this book. Its focus is the metabolism of ceramide in normal and diseased states and the biophysical and biochemical mechanisms governing the bioactivity of these molecules. Sphingolipid research has surged over the past thirty years and this book gathers the recent findings of various aspects of sphingolipid biochemistry. World-renowned scientists from the field of lipid biology, specifically sphingolipid biochemistry, were gathered to write this book. Scholars from most continents of the globe committed to write diligently about their expertise and the newest findings in the relevant fields. This book came to fruition after almost a year and a half of laborious preparation and diligent writings. This book is targeted to the experienced reader who is looking to read about the various aspects of bioactive ceramide signaling, as well as to the newcomer into the field, as the topics are explained in concise yet very informative manner. The authors and editor wish all readers a pleasant time reading this volume, and are adamant that this book will meet all expectations.




Regulation of Hepatic Metabolism


Book Description

The liver is an exceptionally complex and diverse organ that functions both as an exocrine and an endocrine gland. It secretes bile, which contains many con stituents in addition to bile salts, and it synthesizes and releases many substances in response to the body's demands, including prohormones, albumin, clotting factors, glucose, fatty acids, and various lipoproteins. It has a dual blood supply providing a rich mixture of nutrients and other absorbed substances via the portal vein and oxygen-rich blood via the hepatic artery. This functional heterogeneity is accompanied by cellular heterogeneity. The liver contains many cell types including hepatic parachymal cells, Kiipffer cells, Ito cells, and endothelial cells. The most abundant cell type, the parenchymal cells, are biochemically and structurally heterogeneous. The cells in the oxygen-rich areas of the portal triad appear more dependent on oxidative metabolism, whereas those around the central vein (pericentral, perivenous, or centrolobular areas) are more dependent upon an anaerobic mechanism. Throughout this volume the latter three terms are used synonymously by various authors to indicate the five to eight layers of cells radiating from the central vein. Structural and metabolic heterogeneity of hepatic parenchymal cells has been demonstrated by a variety of approaches, including histochemical, ultra structural, and ultramicrobiochemical studies. This microheterogeneity is linked to the physiological functions of the liver and its response to injurious substances.




Lipids in Health and Disease


Book Description

Lipids are functionally versatile molecules. They have evolved from relatively simple hydrocarbons that serve as depot storages of metabolites and barriers to the permeation of solutes into complex compounds that perform a variety of signalling functions in higher organisms. This volume is devoted to the polar lipids and their constituents. We have omitted the neutral lipids like fats and oils because their function is generally to act as deposits of metabolizable substrates. The sterols are also outside the scope of the present volume and the reader is referred to volume 28 of this series which is the subject of cholesterol. The polar lipids are comprised of fatty acids attached to either glycerol or sphingosine. The fatty acids themselves constitute an important reservoir of substrates for conversion into families of signalling and modulating molecules including the eicosanoids amongst which are the prostaglandins, thromboxanes and leucotrienes. The way fatty acid metabolism is regulated in the liver and how fatty acids are desaturated are subjects considered in the first part of this volume. This section also deals with the modulation of protein function and inflammation by unsaturated fatty acids and their derivatives. New insights into the role of fatty acid synthesis and eicosenoid function in tumour progression and metastasis are presented.




Endocrine Disrupting Chemicals-induced Metabolic Disorders and Treatment Strategies


Book Description

This volume offers a detailed and comprehensive analysis of Endocrine Disrupting Chemicals (EDCs), covering their occurrence, exposure to humans and the mechanisms that lead to the parthogenesis of EDCs-induced metabolic disorders. The book is divided into three parts. Part I describes the physiology of the human endocrine system, with special emphasis on various types of metabolic disorders along with risk factors that are responsible for the development of these disorders. Part II addresses all aspects of EDCs, including their role in the induction of various risk factors that are responsible for the development of metabolic disorders. Part III covers up-to-date environmental regulatory considerations and treatment strategies that have been adopted to cure and prevent EDCs-induced metabolic disorders. This section will primarily appeal to clinicians investigating the causes and treatment of metabolic disorders. The text will also be of interest to students and researchers in the fields of Environmental Pharmacology and Toxicology, Environmental Pollution, Pharmaceutical Biochemistry, Biotechnology, and Drug Metabolism/Pharmacokinetics.