The most recent developments in research on hepatic encephalopathy, inborn hyperammonaemic syndromes and nitrogen metabolism, including clinical aspects, were presented by international acknowledged experts in this active research field at the 12th International Symposium on Hepatic Encephalopathy, which was held from June 1 to 4, 2005 in Solingen, Schloss Burg, Germany. This book comprehensively summarizes the most important novel issues on hepatic encephalopathy and nitrogen metabolism and is of interest not only for scientists in the field, but also for interested clinicians. This update of present knowledge will provide a platform for future research in the field of hepatic encephalopathy and nitrogen metabolism.

Amino acid transport is a part of each of two larger subjects, amino acid metabolism and the biomembrane transport of various . small molecules and ions. Nevertheless in this volume we treat amino acid transport as more than a fragment of either of these two larger subjects. A more comprehensive approach is justified when we remember two historic and ongoing aspects of the title subject. First, amino acid transport had its beginning and acquired a distinct momentum (even if somewhat interrupted from 1913 until about 1945) as amino acid metabolism with the central and pioneer work of Van Slyke and Meyer in 1913. The reviews in this volume will show that it steadily becomes a larger aspect of amino acid metabolism, broadly perceived. These chapters will show for how many organelles, cells, tissues, organs and organ systems, the transmembrane compartmentations and flows of amino acids play very large parts in their fundamental biological relations. The authors here are tending collectively to evaluate an understanding of amino acid flows across biomernbranes, and the regulation of these flows, as necessary to an ultimate understanding of the full range of development and metabolism. Such an understanding goes far beyond the purely substrate-destabilizing contributions by enzymes, which have often been arbitrarily limited to that conceptual entity, "the cell," and which for so long a splendid time had most of biochemical research attention.

This book is the proceedings of the Falk Symposium No.156 on ?Genetics in Liver Disease?, part of the XIII International Liver Week 2006 held in Freiburg, Germany, 7 October 2006. The first section covers the basic aspects of genetic diagnosis, pharmacogenetics, micro-arrays and their relevance for liver diseases, including viral hepatitis, hepatocellular carcinoma, and gallstone diseases. In the second section, the most important hereditary liver diseases are discussed, including haemchromatosis, Wilson disease, alpha-1-antitrypsin deficiency, porphyrias and cystic fibrosis. In the third section, the genetics of cholestatic and metabolic liver diseases as well as the current status of experimental and clinical studies of gene therapy for liver diseases and stem cell transplantation are presented. Each section starts with a State-of-the-Art Lecture which introduces the topic. In the tradition of the Falk Symposia, this book provides an exciting overview of the current developments in the field of genetics of liver diseases, their diagnosis, treatment and prevention, presented by an international array of outstanding scientists and clinicians.

Hepatic Encephalopathy and Nitrogen Metabolism is an interdisciplinary symposium bringing together basic science and clinical applications. It contains up-to-date research findings at the highest scientific level.

Amino acid transport is a part of each of two larger subjects, amino acid metabolism and the biomembrane transport of various . small molecules and ions. Nevertheless in this volume we treat amino acid transport as more than a fragment of either of these two larger subjects. A more comprehensive approach is justified when we remember two historic and ongoing aspects of the title subject. First, amino acid transport had its beginning and acquired a distinct momentum (even if somewhat interrupted from 1913 until about 1945) as amino acid metabolism with the central and pioneer work of Van Slyke and Meyer in 1913. The reviews in this volume will show that it steadily becomes a larger aspect of amino acid metabolism, broadly perceived. These chapters will show for how many organelles, cells, tissues, organs and organ systems, the transmembrane compartmentations and flows of amino acids play very large parts in their fundamental biological relations. The authors here are tending collectively to evaluate an understanding of amino acid flows across biomernbranes, and the regulation of these flows, as necessary to an ultimate understanding of the full range of development and metabolism. Such an understanding goes far beyond the purely substrate-destabilizing contributions by enzymes, which have often been arbitrarily limited to that conceptual entity, "the cell," and which for so long a splendid time had most of biochemical research attention.

One of the prerequisites for survival is the ability of cells to maintain their volume. Thus, during the course of evolution cells have "learned" a variety of strategies to achieve volume homeostasis. This volume regulatory machinery involves regulation of both, cellular metabolism and cellular transport and is exploited by hormones and transmitters to regulate cellularfunction. This book to illustrates the complex interplay of cell volume regulatory mechanisms and cellular function in a variety of tissues. However, our knowledge is still far from being conclusive, and the present collection of reviews is thought to foster further experimental efforts to unravel the role of cell volume in the integrated function of cells.

Glutamine is a key aminoacid for the synthesis of numerous biologic- ally important compounds in mammalian cells and is a carrier form of ammonia. The advance in knowledge on the metabolic significance of this amino acid is presented in in-depth treatments by experts in this active research field. This includes the enzymology of glutamine synthe- tase and glutaminase activities in different mammalian organs, notably liver, kidney and brain; properties of glutamine transport across bio- logical membranes; role of glutamine metabolism in the liver, with emphasis on the recent discovery of intercellular heterogeneity with respect to enzyme distribution and its functional consequences for ammonia/urea metabolism and pH regulation; renal and intestinal glutamine metabolism; cerebral glutamine/ glutamate interrelationships; skeletal muscle; role of glutamine in cell culture; and finally the clinical aspects, including the new outlook of glutamine antagonists in cancer therapy as well as the role of glutamine in hepatic coma and encephalo- pathy. Some, but not all, of the contributors to this work attended the 48th Conference of the Gesellschaft fur Biologische Chemie on glutamine metabolism held in Gottingen, W. Germany, in September 1983. This conference was supported by the Stiftung Volkswagenwerk, Dr. H. Falk Foundation, Freiburg, and the J. Pfrimmer Co. , Erlangen. The abstracts of the contributions to the conference were published in Hoppe-Seyler's Z. Physiol. Chem. 364,1237-125,6,1983, and this book is not intended as the proceedings of that meeting.