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.

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.

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.

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.

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 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. ​

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. ​

Biochemistry of Lipids: Lipoproteins and Membranes, Volume Six, contains concise chapters that cover a wide spectrum of topics in the field of lipid biochemistry and cell biology. It provides an important bridge between broad-based biochemistry textbooks and more technical research publications, offering cohesive, foundational information. It is a valuable tool for advanced graduate students and researchers who are interested in exploring lipid biology in more detail, and includes overviews of lipid biology in both prokaryotes and eukaryotes, while also providing fundamental background on the subsequent descriptions of fatty acid synthesis, desaturation and elongation, and the pathways that lead the synthesis of complex phospholipids, sphingolipids, and their structural variants. Also covered are sections on how bioactive lipids are involved in cell signaling with an emphasis on disease implications and pathological consequences. - Serves as a general reference book for scientists studying lipids, lipoproteins and membranes and as an advanced and up-to-date textbook for teachers and students who are familiar with the basic concepts of lipid biochemistry - References from current literature will be included in each chapter to facilitate more in-depth study - Key concepts are supported by figures and models to improve reader understanding - Chapters provide historical perspective and current analysis of each topic

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.

The 2nd Edition of Metabolic Diseases provides readers with a completely updated description of the Foundations of Clinical Management, Genetics, and Pathology. A distinguished group of 31 expert authors has contributed 25 chapters as a tribute to Enid Gilbert-Barness and the late Lewis Barness--- both pioneers in this topic. Enid’s unique perspectives on the pathology of genetic disorders and Lew’s unsurpassed knowledge of metabolism integrated with nutrition have inspired the contributors to write interdisciplinary descriptions of generally rare, and always challenging, hereditary metabolic disorders. Discussions of these interesting genetic disorders are organized in the perspective of molecular abnormalities leading to morphologic disturbances with distinct pathology and clinical manifestations. The book emphasizes recent advances such as development of improved diagnostic methods and discovery of new, more effective therapies for many of the diseases. It includes optimal strategies for diagnosis and information on access to specialized laboratories for specific testing. The target audience is a wide variety of clinicians, including pediatricians, neonatologists, obstetricians, maternal-fetal specialists, internists, pathologists, geneticists, and laboratorians engaged in prenatal and/or neonatal screening. In addition, all scientists and health science professionals interested in metabolic diseases will find the comprehensive, integrated chapters informative on the latest discoveries. It is our hope that the 2nd Edition will open new avenues and vistas for our readers and that they will share with us the interest, excitement and passion of the research into all these challenging disorders.