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The peroxisome is an organelle with essential roles in lipid metabolism, maintenance of reactive oxygen species homeostasis, and anaplerotic replenishment of tricarboxylic acid cycle intermediates destined for mitochondria. Peroxisomes constitute a dynamic endomembrane system. The homeostatic state of this system is upheld via two pathways for assembling and maintaining the diverse peroxisomal compartments constituting it; the relative contribution of each pathway to preserving such system may vary in different organisms and under various physiological conditions. One pathway begins with the targeting of certain peroxisomal membrane proteins to an endoplasmic reticulum template and their exi...
In most peroxisomal disorders the nervous system is severely affected which explains the clinical and community burden they represent. This is the first book to focus not only on the mutations causing these inherited illnesses, but also on mechanisms that regulate, suppress or enhance expression of genes and their products (enzymes). Indeed since the success and completion of the Human Genome Project all genes (coding DNA sequences) are known. However, of many, their function, and the role of the gene product has not been determined. An example is X-linked adrenoleukodystrophy, the most frequent peroxisomal disorder. Children are born healthy, but in more than 1 out of 3, demyelination of the brain starts unpredictably and they die in a vegetative state. The gene mutated in most families has been known for 10 years; but the true role of the encoded protein, ALDp, is still speculative; and within the same family, very severe and asymptomatic clinical histories co-exist, unexplained by the mutation.
This book contains the proceedings of the XVIII International Symposium on Retinal Degeneration (RD2018). A majority of those who spoke and presented posters at the meeting contributed to this volume. The blinding diseases of inherited retinal degenerations have no treatments, and age-related macular degeneration has no cures, despite the fact that it is an epidemic among the elderly, with 1 in 3-4 affected by the age of 70. The RD Symposium focused on the exciting new developments aimed at understanding these diseases and providing therapies for them. Since most major scientists in the field of retinal degenerations attend the biennial RD Symposia, they are known by most as the “best” a...
It is thirty years since the technique of high-yield preparation of isolated hepatocytes, by collagenase perfusion of the liver, was published. The original method described by Berry and Friend has undergone many minor modifications by other workers, and the two-step procedure introduced by Seglen in 1976 has become the most frequent way to prepare hepatocyte suspensions. An important development introduced by Bissell in 1973 was the use of the cells as the first step in monolayer culture. The availability of the isolated hepatocyte preparation as cells in suspension or culture has undoubtedly facilitated research on the liver. This was emphasised in our book, published (with Dr. Greg Barrit...
WHAT HAPPENED IN KANAZAWA? THE BIRTH OF eCAM This book contains the proceedings of the International Symposium on Complementary and Alternative Medicine, (CAM) which was convened in Kanazawa Japan, November 8-10, 2002. The participants were mainly from Japan, USA, China, France, England, Germany, Taiwan, and India. The world of western medicine is gradually opening its doors to new ways of ap proaching healing. Since many of these approaches began centuries and even millennia ago in Asia, it was entirely appropriate to open our symposium in Kanazawa, a beautiful, traditional city located on the Sea of Japan. Experts from Asia, Europe and the United States gathered together for true discussio...
In eukaryotes, lipid metabolism requires the function of peroxisomes. These multitasking organelles are also part of species-specific pathways such as the glyoxylate cycle in yeast and plants or the synthesis of ether lipid in mammals. Proteins required for the biogenesis of peroxisomes typically assemble in large molecular complexes, which participate in membrane formation, protein transport, peroxisome duplication and - inheritance during cell division. Peroxisomal function is essential for life. Mutations in PEX genes, encoding for biogenesis factors, are often associated with lethal disorders. The association of peroxisomes with other organelles suggests an extensive participation in organellar crosstalk. This book represents a state-of-the-art review in the field of peroxisome research encompassing the cell and molecular biology of peroxisome biogenesis and its diseases, the protein complexes involved in this process and the modern technologies applied to study them. The book is intended for graduate students, researchers and lecturers in biochemistry, molecular and cell biology with a biomedical background.
Post Genomic Perspectives in Modeling and Control of Breathing is comprised of the proceedings of the IXth Oxford Conference on Modeling and Control of Breathing, held September 13-16, 2003 in Paris, France. This publication is placed within the general framework of post-genomic neurobiology, pathology, and the precise example of the rhythmic respiratory neural assembly being used to understand how genetic networks have been selected and conserved in the vertebrate brain. Specific topics include: ion channels and synapses responsible for respiratory rhythmogenesis and plasticity; pre- and post-natal development of the respiratory rhythm; chemosensory transduction and chemo-afferent signalling. These valuable insights open new avenues as to why the genetic codes underlying a vital function such as breathing have been selected, conserved, or optimized during evolution – a major issue of post-genomic biology. This critical issue will be considered from both top-down and bottom-up integrative modeling standpoints, with a view to elucidating the functional genomics linking discrete molecules to the integrated system that regulates breathing.
The International Hypoxia Symposium convenes biannually to bring together international experts from many fields to explore the state of the art in normal and pathophysiological responses to hypoxia. Representatives from five continents and 32 countries joined together in February 2003 for four days in the dramatic mountains of Banff, Alberta. As editors of the Proceedings of the International Hypoxia Symposia, we strive to maintain a 26 six year tradition of presenting a stimulating blend of clinical and basic science papers focused on hypoxia. Topics covered in 2003 include hibernation and hypoxia, hypoxia and fetal development and new advances in high altitude pathophysiology, oxidative stress and membrane damage, hypoxic regulation of blood flow, heat shock proteins in hypoxia, and future directions in hypoxia research. In 2003 we also had the privilege ofhonoring John W. Severinghaus as a friend, colleague, mentor and inspiration to many in the field. Tom Hornbein's personal tribute to John Severinghaus is the first chapter in this volume, followed by an entertaining update of the history of the discovery of oxygen written by John Severinghaus.
This book is based on the proceedings of the inaugural symposium "Northwest Symposium for Systems Biology". Particular focus is on identifying current breakthrough technologies and their application to important model systems. By integrating computational sciences, high-throughput technologies and quantitative biology, this book will advance the understanding of not just the function of individual genes, proteins and smaller molecules like hormones, but also how all of these molecules interact within a cell. This volume will be a collection of presentations on four topics that comprise the scope of the Genomes to Life project recently announced by the Department of Energy. They are four aspects of a system biology approach to understanding microbial communities. These topics include complex microbial systems, gene regulatory networks, molecular machines/ multiprotein complexes and computational techniques.