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I was gratified by the most favorable reception and wide usage received by the first edition of this book. A decade seems to be a short period for a book on pathology, and yet it witnessed many important changes of concepts, along with a formidable growth of knowledge. The second edition required extensive reorganization. There are new chapters on mitochondriopathies, on peroxisomal diseases and on spongy myelino pathies. Major revisions and new additions were necessary in many chapters, for instance those on the dysplasias of the cerebral and of the cerebellar hemispheres, which were largely reorganized. The chapters on perinatal pathology were reordered and reorganized to give a more logical sequence of prenatal, perinatal and postnatal lesions. The entire text was worked over for brevity. A wealth of new references was added with the. aim of staying abreast with the literature up to summer 1988. All refer ences were double checked for errors. My gratitude goes to Mrs. Gisela Ropte and Mrs. Cynthia Bunker for their untiring, diligent help. As a result, this second edition is an essentially rewritten text. Advance in the prevention of human suffering is based on a thorough understand ing of the nature of disease. I hope that this text will continue to be of service in this behalf. Perhaps it may also reflect and foster the intellectual curiosity which makes the "reading of brains" so interesting an occupation. Gottingen, 1989 Reinhard L.

This volume provides state-of-the-art techniques for studying various aspects of cholesterol homeostasis, including its uptake, synthesis and efflux from the cell, as well as its trafficking within the cell. Chapters also cover techniques for studying the regulation of cholesterol homeostasis at both the transcriptional and post-translational levels, as well as studying the membrane topology and structure of cholesterol-related proteins. Written in the highly successful Methods in Molecular Biology series format, chapters include introductions to their respective topics, lists of the necessary materials and reagents, step-by-step, readily reproducible laboratory protocols, and tips on troubleshooting and avoiding known pitfalls. Authoritative and practical, Cholesterol Homeostasis: Methods and Protocols aims to provide key techniques in tackling the investigation of cholesterol homeostasis.

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.

Multivariate analysis of the multi-component analytical profiles of carefully collected biofluid and/or tissue biopsy specimens can provide a 'fingerprint' of their biomolecular/metabolic status. Therefore, if applied correctly, valuable information regarding disease indicators, disease strata and sub-strata and disease activities can be obtained. This exemplary new book highlights applications of these techniques in the areas of drug therapy and toxicology, cancer, obesity and diabetes, as well as outlining applications to cardiovascular, infectious, inflammatory and oral diseases in detail. The book gives particular reference to cautionary measures that must be applied to the diagnosis and classification of these conditions or physiological criteria. Comprehensively covering a wide range of topics, of particular interest is the focus on experimental design and 'rights and wrongs' of the techniques commonly applied by researchers, and the very recent development of powerful 'Pattern Recognition' techniques. The book provides a detailed introduction to the area, applications and common pitfalls of the techniques discussed before moving into detailed coverage of specific disease areas, each highlighted in individual chapters. This title will provide an invaluable resource to Medicinal chemists, Biochemists and toxicologists working in industry and academia.

A review of childhood neurodegenerative and other progressive but non-degenerative disorders to guide their diagnosis and management.

Covers the most frequently asked and tested points on the pediatric board exam. Each chapter offers a quick review of specific diseases and conditions clinicians need to know during the patient encounter. Easy-to-use and comprehensive, clinicians will find this guide to be the ideal final resource needed before taking the pediatric board exam.