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Book Sphingolipids  Sphingolipidoses and Allied Disorders

Download or read book Sphingolipids Sphingolipidoses and Allied Disorders written by Bruno Volk and published by Springer Science & Business Media. This book was released on 2013-04-17 with total page 693 pages. Available in PDF, EPUB and Kindle. Book excerpt: This text contains the scientific contributions to the Fourth International Symposium on Sphingolipids, Sphingo lipidoses and Allied Disorders held at the Kingsbrook Jewish Medical Center on October 25-27, 1971. These meetings were conducted under the auspices of the Isaac Albert Research Institute of the Kingsbrook Jewish Medical Center and the National Tay-Sachs and Allied Diseases Association, Inc. Four symposia, held in 1958, 1961, 1965 and 1971 were designed to gather the most relevant and innovative of the laboratory and field studies concerned with these hereditary disorders. The texts generated by these periodic meetings have mirrored the increasing absorption of the scientific community in the problems of sphingolipid metabolism. The first meeting in 1958 consisted of but twelve pre sentations, the majority emanating from local laboratories. The current sessions contain 48 scientific presentations by scientists from nine countries and demonstrate the increas ingly diversified techniques and approaches employed in the study of these diseases. Many of the authors, in exploring data on the mucopolysaccharidoses and leucodystrophies, as well as the sphingolipidoses, have given recognition to those biochemical areas held in common by these otherwise diverse disease processes. The problems of prevention and therapy of these diseases have been considered by some of the contributors. Laboratory screening procedures designed to detect carriers of the va rious lipidoses are now available and the experiences of some laboratories in this area are summarized within this volume. The prospective identification of heterozygotes may indeed become a powerful adjunct in genetic counseling.

Book Sphingolipids  Sphingolipidoses and Allied Disorders

Download or read book Sphingolipids Sphingolipidoses and Allied Disorders written by International Symposium on the Cerebral Sphingolipidoses, 4th, Kingsbrook Jewish Medical Center, 1971 and published by . This book was released on 1972 with total page 691 pages. Available in PDF, EPUB and Kindle. Book excerpt:

Book Sphingolipids  Sphingolipidoses and Allied Disorders

Download or read book Sphingolipids Sphingolipidoses and Allied Disorders written by Bruno Volk and published by Springer. This book was released on 2014-01-15 with total page 720 pages. Available in PDF, EPUB and Kindle. Book excerpt:

Book Inborn Disorders of Sphingolipid Metabolism

Download or read book Inborn Disorders of Sphingolipid Metabolism written by Stanley M. Aronson and published by Elsevier. This book was released on 2017-01-31 with total page 530 pages. Available in PDF, EPUB and Kindle. Book excerpt: 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.

Book Current Trends in Sphingolipidoses and Allied Disorders

Download or read book Current Trends in Sphingolipidoses and Allied Disorders written by Bruno Volk and published by Springer Science & Business Media. This book was released on 2013-03-09 with total page 603 pages. Available in PDF, EPUB and Kindle. Book excerpt: The present volume contains the scientific contributions to the Fifth International Symposium on "Current Trends in Sphingo lipidoses and Allied Disorders" under the auspices of the Isaac Albert Research Institute of the Kingsbrook Jewish Medical Center, the Department of Pathology, Downstate Medical Center, State Uni versity of New York, Brooklyn, New York, and the National Tay-Sachs and Allied Diseases Association, Inc., New York. A review of the four previous Symposia shows the increase in scope of the scientific exploration in this rapidly expanding field. The first meeting, held in 1958, was devoted to the discussion al most entirely of Tay-Sachs disease. The majority of the work emanated from local laboratories. The participants at the present Symposium came from many other domestic and foreign research in stitutions. The scope of the papers presented at these meetings and the interest shown in the Symposium demonstrates the signifi cance attached by the scientific community to the problems of these hereditary diseases. The reasons for this are apparent, when one considers the contributions during recent years to our basic know ledge by lipid and enzyme chemistry, genetics, and neuropathology. Partly because of the hereditary nature of these diseases any new discovery in this field has general meaning and permits cautious generalization well beyond its clinical significance.

Book Sphingolipidoses and Allied Disorders

Download or read book Sphingolipidoses and Allied Disorders written by M. Adachi and published by . This book was released on 1979 with total page 260 pages. Available in PDF, EPUB and Kindle. Book excerpt:

Book Cerebral Sphingolipidoses

Download or read book Cerebral Sphingolipidoses written by Stanley M. Aronson and published by Elsevier. This book was released on 2013-10-22 with total page 475 pages. Available in PDF, EPUB and Kindle. Book excerpt: Cerebral Sphingolipidoses: A Symposium on Tay-Sach’s Disease and Allied Disorders is a collection of papers presented at the 1961 Symposium on the Cerebral Sphingolipidoses, held in Isaac Albert Research Institute of the Jewish Chronic Disease Hospital and the Downstate Medical Center of the State University of New York. The contributors demonstrate the significance attached to an intensive study of Tay-Sach’s and allied diseases. A unique feature of the Symposium was the constant awareness of the clinical implications of the fundamental studies presented. This book is organized into three sections encompassing 30 chapters. The first part deals with the morphological aspects of cerebral lipidoses, including the diagnosis, biopsy, pathology, and clinical features of these diseases. This part also provides case reports of specific cerebral sphingolipidoses. The second part highlights the biochemical aspects of Tay-Sach’s and allied disorders. This part specifically looks into the mechanism of abnormal lipid metabolism, chemical pathology of lipids, and quantitative fractionation of complex lipid mixtures. This part also considers the characterization of accumulated gangliosides in brain, which is one the most striking features in infantile amaurotic idiocy, a type of Tay-Sach’s disease. The third part explores the genetic and therapeutic aspects of the sphingolipidoses. This book will be of value to lipid chemists, geneticists, biochemists, neuropathologists, and clinicians.

Book Sphingolipidoses and Allied Disorders

Download or read book Sphingolipidoses and Allied Disorders written by and published by . This book was released on 1979 with total page 282 pages. Available in PDF, EPUB and Kindle. Book excerpt:

Book Sphingolipid Biochemistry

    Book Details:
  • Author : Julian N. Kanfer
  • Publisher : Springer Science & Business Media
  • Release : 2012-12-06
  • ISBN : 1475703961
  • Pages : 495 pages

Download or read book Sphingolipid Biochemistry written by Julian N. Kanfer and published by Springer Science & Business Media. This book was released on 2012-12-06 with total page 495 pages. Available in PDF, EPUB and Kindle. Book excerpt:

Book National Library of Medicine Current Catalog

Download or read book National Library of Medicine Current Catalog written by National Library of Medicine (U.S.) and published by . This book was released on 1972 with total page pages. Available in PDF, EPUB and Kindle. Book excerpt: First multi-year cumulation covers six years: 1965-70.

Book The Gangliosidoses

    Book Details:
  • Author : Bruno Volk
  • Publisher : Springer Science & Business Media
  • Release : 2012-12-06
  • ISBN : 1461587263
  • Pages : 388 pages

Download or read book The Gangliosidoses written by Bruno Volk and published by Springer Science & Business Media. This book was released on 2012-12-06 with total page 388 pages. Available in PDF, EPUB and Kindle. Book excerpt: The history of so-called storage diseases goes back to the end of the 19th and to the beginning of the 20th century when Fabry, Tay, Sachs, Gaucher, Niemann, Hunter, and Hurler first described the disorders which up to now are called by their eponym. The clinical descriptions soon were followed by pathologic studies, and within a short time ,the hereditary characters of these rare afflictions came to be recognized. Although sporadic reports during the early part of this century dealt with biochemical analysis of the "stored" materials in these disorders, it was actually in the late 1930s that the abnormal deposits started to attract the increasing attention of chemists. S. H. Thannhauser brought the broad concept of lipidoses as a group of related disorders to the attention of the medical profession for the first time, and in 1939 Klenk observed that the brain of a patient with Tay-Sachs disease contained greatly increased amounts of a glycolipid for which he proposed the name "ganglioside. " 20 years has thrown new light on these afflic Work carried out in the past tions and has pinpointed the enzymatic and lipid abnormalities associated with the various "storage" diseases. Moreover, electron microscopic studies have permitted detailed investigations of the fine structure of the various organs of afflicted patients.

Book Lipid Transfer in Lipoprotein Metabolism and Cardiovascular Disease

Download or read book Lipid Transfer in Lipoprotein Metabolism and Cardiovascular Disease written by Xian-Cheng Jiang and published by Springer Nature. This book was released on 2020-07-23 with total page 235 pages. Available in PDF, EPUB and Kindle. Book excerpt: 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.

Book Biology of Brain Dysfunction

    Book Details:
  • Author : Gerald E. Gaull
  • Publisher : Springer Science & Business Media
  • Release : 2012-12-06
  • ISBN : 1468426702
  • Pages : 431 pages

Download or read book Biology of Brain Dysfunction written by Gerald E. Gaull and published by Springer Science & Business Media. This book was released on 2012-12-06 with total page 431 pages. Available in PDF, EPUB and Kindle. Book excerpt: The growth of neurochemistry, molecular biology, and biochemical genetics has led to a burgeoning of new information relevant to the pathogenesis of brain dysfunction. This explosion of exciting new information is crying out for collation and meaningful synthesis. In its totality, it defies systematic summa tion, and, of course, no one author can cope. Thus invitations for contributions were given to various experts in areas which are under active investigation, of current neurological interest, and pregnant. Although this project is relatively comprehensive, by dint of size, other topics might have been included; the selection was solely my responsibility. I believe systematic summation a virtual impossibility-indeed, hardly worth the effort. The attempt to assemble all of the sections involved in a large treatise with mUltiple authors inevitably results in untoward delays due to the difference in the rate at which various authors work. Therefore, the following strategy has been adopted: multiple small volumes and a relatively flexible format, with publication in order of receipt and as soon as enough chapters are assembled to make publication practical and economical. In this way, the time lag between the ideas and their emergence in print is the shortest.

Book Mental Health Research Institute Staff Publications

Download or read book Mental Health Research Institute Staff Publications written by University of Michigan. Mental Health Research Institute and published by UM Libraries. This book was released on 1975 with total page 528 pages. Available in PDF, EPUB and Kindle. Book excerpt:

Book Advances in Neurochemistry

    Book Details:
  • Author : B. W. Agranoff
  • Publisher : Springer Science & Business Media
  • Release : 2013-11-21
  • ISBN : 1475743955
  • Pages : 317 pages

Download or read book Advances in Neurochemistry written by B. W. Agranoff and published by Springer Science & Business Media. This book was released on 2013-11-21 with total page 317 pages. Available in PDF, EPUB and Kindle. Book excerpt: The emergence of a new scientific book series requires some explanation regarding how it hopes to compensate the reader for the discomforts it undoubtedly produces both in the realms of informational input-overload and in the financial strain on personal and institutional budgets. This series recognizes that investigators who have entered neurochemistry from the biochemical tradition have a rather specialized view of the brain. Too often, interdisciplinary offerings are initially attractive but turn out to recite basic biochemical considerations. We have come to believe that there are now sufficiently large numbers of neurochemists to support a specialized venture such as the present one. We have begun with consideration of traditional areas of neurochemistry which show considerable scientific activity. We hope they will serve the neurochemist both for general reading and for specialized information. The reader will also have the opportunity to reftect on the unbridled speculation that results from the disinhibiting effects on the author who has been invited to write a chapter. We plan occasionally also to offer reviews of areas not completely in the domain of neurochemistry which we nevertheless feel to be sufficiently timely to be called to the attention of all who use chemical principles and tools in an effort to better understand the brain. B. W. Agranoff M. H. Aprison vii CONTENTS CHAPTER 1 POSSIBLE ROLES OF PROSTAGLANDINS IN THE NERVOUS SYSTEM LEONHARD S. WOLFE 1. Introduction 1 1. 1. Background 1 Names and Structures 1. 2. 4 1. 3. Biosynthesis 4 1. 4.

Book Genetic Aberrancies and Neurodegenerative Disorders

Download or read book Genetic Aberrancies and Neurodegenerative Disorders written by M.P. Mattson and published by Elsevier. This book was released on 2000-01-28 with total page 427 pages. Available in PDF, EPUB and Kindle. Book excerpt: Genetic Aberrancies and Neurodegenerative Disorders presents critical reviews and emerging findings concerning the roles of genetic mutations and polymorphisms in the pathogenesis of a range of neurological disorders including Alzheimer's and Parkinson's diseases, trinucleotide repeat disorders, stroke, epilepsy and others. This volume, written by leading experts, brings together fundamental information concerning the roles of inherited traits in the pathogenesis of different neurodegenerative disorders. In addition to providing a catalogue of the known genetic alterations that are linked to specific neurodegenerative disorders, the chapters detail the current state of understanding of the cellular and biochemical mechanisms whereby the genetic aberrancies lead to neuronal dysfunction and degeneration. The emerging picture of each disorder, painted by pathological, biochemical and molecular brushes, suggests that they share key mechanisms including increased levels of oxidative stress, perturbed ion homeostasis, mitochondrial dysfunction and apoptotic proteolytic cascades. The existence of specific molecular defects provides the opportunity to design experiments that can establish the precise pathogenic mechanism of a specific mutation or genetic risk factor. The value of this approach is exemplified by recent studies of how mutations in Cu/Zn-superoxide dismutase cause amyotrophic lateral sclerosis and how presenilin mutations result in early-onset Alzheimer's disease. A theme developed among the different chapters is that events that occur during aging predispose neurons to genetic aberrancies that promote degenerative cascades, and that specific genetic defects exert their influence on particular populations of neurons in a disorder-specific manner. The chapters in this volume will stimulate readers to generate new hypotheses concerning the pathogenic mechanisms of genetic aberrancies that can be experimentally tested.

Book Biological Roles of Sialic Acid

Download or read book Biological Roles of Sialic Acid written by Abraham Rosenberg and published by Springer Science & Business Media. This book was released on 2013-11-11 with total page 386 pages. Available in PDF, EPUB and Kindle. Book excerpt: There is a startling amount of research activity concerning the role of sialic acid in mammalian cells and in the mammalian organism. One may discern in the early literature premonitions of compounds containing sialic acid, traceable by descriptions of color reactions, as far back as the turn of the century. Work spanning the 1930s to the 1950s culmi nated in the crystallization of sialic acid from a wide variety of biological materials. The ubiquitous nature of the sialic acids, and the biological importance of the substances in which they occur, then became generally manifest. Since then, the chemistry and metabolism of sialic acid and its occurrence, notably, but not exclusively, in the outer cell surfaces of mammalian cells and in key extracellular glycoproteins, have received great attention. The involvement of sialic acid-containing substances in tumorigenicity and in numerous metabolic and infectious pathological conditions, and in the growth, development, and integrity of mammalian cells has achieved widespread recognition. Intensive inquiry into the biological roles of sialic acid continues in a large number of research laboratories throughout the world. This book is intended to represent for the uninitiated as well as for the expert a wide and detailed overview of the current state of knowledge. Major efforts and pioneering break throughs have emerged from several laboratories, located on both sides of the Atlantic, of which we make no special individual mention here since they will to some extent appear in the pages that follow.