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.

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.

As clinical management of inherited metabolic diseases (IMDs) has improved, more patients affected by these conditions are surviving into adulthood. This trend, coupled with the widespread recognition that IMDs can present differently and for the first time during adulthood, makes the need for a working knowledge of these diseases more important than ever. Inherited Metabolic Disease in Adults offers an authoritative clinical guide to the adult manifestations of these challenging and myriad conditions. These include both the classic pediatric-onset conditions and a number of new diseases that can manifest at any age. It is the first book to give a clear and concise overview of how this group of conditions affects adult patients, a that topic will become a growing imperative for physicians across primary and specialized care.

This volume presents information on both the basic and clinical aspects of sphingolipid-metabolizing enzymes in various cancers. The volume also includes discussions of the innovative techniques and approaches for quantitative analysis and imaging that could significantly impact the general understanding of this topic, and the potential benefit of targeting sphingolipid enzymes to develop novel cancer therapeutics. As well, the volume includes a critical examination of the specific pathways and pathobiologies associated with the altered regulation of sphingolipid metabolism as a contributor to the development and/or maintenance of pathological conditions such as cancer.

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.