The writing of this book was prompted by the need for a comprehensive of current data on organic acids suitable for both newcomers and collection established researchers in this field. The only previous text of the kind was the excellent review by Nordmann and Nordmann (1961), and at that time the main method of analysis was paper chromatography with liquid chromatography being used in a limited way. Only three diseases in which organic acids accumulate were known (primary hyperoxaluria, phenylketonuria and alcaptonuria). Since then, with the development of gas chromatography and mass spectrometry, and the further development ofliquid chromatography, knowledge concerning the nature of the organic acids in physiological fluids has been greatly extended. At the same time, the number of organic acidurias has increased dramatically, there being now some 40-50 known diseases of this type. During the past 15 years or so, there have been several reviews, dealing with either specific diseases or groups of diseases (Gompertz, 1972, 1974; Tanaka, 1975), or presenting the proceedings of symposia (Stern and Toothill, 1972) or workshops (Marner et al. , 1974). This present text deals comprehensively and in detail with the organic acids in human physiological fluids in health and in disease states, and is particularly concerned with the methods necessary for their separation, determination and indentification.

A collection of papers that comprehensively describe the major areas of research on lipid metabolism of plants. State-of-the-art knowledge about research on fatty acid and glycerolipid biosynthesis, isoprenoid metabolism, membrane structure and organization, lipid oxidation and degradation, lipids as intracellular and extracellular messengers, lipids and environment, oil seeds and gene technology is reviewed. The different topics covered show that modern tools of plant cellular and molecular biology, as well as molecular genetics, have been recently used to characterize several key enzymes of plant lipid metabolism (in particular, desaturases, thioesterases, fatty acid synthetase) and to isolate corresponding cDNAs and genomic clones, allowing the use of genetic engineering methods to modify the composition of membranes or storage lipids. These findings open fascinating perspectives, both for establishing the roles of lipids in membrane function and intracellular signalling and for adapting the composition of seed oil to the industrial needs. This book will be a good reference source for research scientists, advanced students and industrialists wishing to follow the considerable progress made in recent years on plant lipid metabolism and to envision the new opportunities offered by genetic engineering for the development of novel oil seeds.

Completely revised new edition of the premier reference on pediatric liver disease. Liver Disease in Children, 3rd Edition provides authoritative coverage of every aspect of liver disease affecting infants, children, and adolescents. The book offers an integrated approach to the science and clinical practice of pediatric hepatology and charts the substantial progress in understanding and treating these diseases. Chapters are written by international experts and address the unique pathophysiology, manifestations, and management of these disorders in the pediatric population. The third edition has been thoroughly updated and features new contributions on liver development, cholestatic and autoimmune disorders, fatty liver disease, and inborn errors of metabolism. With the continued evolution of pediatric hepatology as a discipline, this text remains an essential reference for all physicians involved in the care of children with liver disease.

Lipid Modification by Enzymes and Engineered Microbes covers the state-of-the art use of enzymes as natural biocatalysts to modify oils, also presenting how microorganisms, such as yeast, can be designed. In the past ten years, the field has made enormous progress, not only with respect to the tools developed for the development of designer enzymes, but also in the metabolic engineering of microbes, the discovery of novel enzyme activities, and in reaction engineering/process development. For the first time, these advances are covered in a single-volume that is edited by leading enzymatic scientist Uwe Borchscheuer and authored by an international team of experts. - Identifies how, and when, to use enzymes and microbes for lipid modification - Provides enzymatic, microbial and metabolic techniques for lipid modification - Covers lipases, acyltransferases, phospholipases, lipoxygenases, monooxygenases, isomerases and sophorolipids - Includes lipid modification for use in food, biofuels, oleochemicals and polymer precursors

Cellular and Molecular Pathology of the Liver is extensive, complex and ranges from the understanding the basic molecular mechanisms that dictate everything from liver homeostasis to liver disease. Molecular Pathology of the liver is complicated due to some of the important functions inherent and unique to the Liver, including its innate ability to regenerate and the multitude of functions it plays for the wellbeing of an organism. With all this in mind, Molecular Pathology of Liver Diseases is organized in different sections, which will coherently and cohesively present the molecular basis of hepatic physiology and pathology. The first two sections are key to understanding the liver anatomy and physiology at a cellular level and go on to define the molecular mechanics in various liver cell types. These sections also cover the existing paradigms in liver development, regeneration and growth. The next section is key to understanding the Molecular Pathology unique to liver diseases and associated phenotypes. The final sections are geared towards the existing knowledge of the molecular basis of many common and uncommon liver diseases in both neoplastic and non-neoplastic areas including pathologies associated with intra-hepatic and extra-hepatic biliary tree. Thus, this textbook is a one-stop reference for comprehending the molecular mechanisms of hepatic pathobiology. It is clearly unique in its format, readability and information and thus will be an asset to many in the field of Pathology and other disciplines.