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This book provides an overview of the biology and biochemistry of peroxisomes, and discusses the contribution of these organelles to peroxisomal and neurodegenerative diseases. It begins with a detailed introduction to the biogenesis and metabolic functions of peroxisomes, and highlights their role in oxidative stress and in lipid metabolism such as fatty acid oxidation. The following chapters focus on the molecular and clinical aspects of peroxisomal disorders caused by defects in peroxisomal function. In particular, the biological aspects of peroxisomal biogenesis disorders such as Zellweger syndrome and Heimler syndrome are discussed. This includes their underlying genetic causes as well as the biochemical and metabolic defects associated with the disorders. In addition, several chapters cover recent observations suggesting an association between peroxisomal dysfunction and neurodegenerative diseases such as Alzheimer's, Multiple Sclerosis and other degenerative cerebellar pathologies. The final section of the book discusses important cell and animal models for studying the role of peroxisomes in human diseases and presents current therapeutic strategies for their treatment. This book deals with a highly topical subject that is at the heart of current research, and represents a valuable contribution for all students and researchers who want to understand the complex biology of peroxisomes and their role in human diseases.
In the two decades since the last comprehensive work on plant peroxisomes appeared, the scientific approaches employed in the study of plant biology have changed beyond all recognition. The accelerating pace of plant research in the post-genomic era is leading us to appreciate that peroxisomes have many important roles in plant cells, including reserve mobilisation, nitrogen assimilation, defence against stress, and metabolism of plant hormones, which are vital for productivity and normal plant development. Many plant scientists are finding, and will no doubt continue to find, that their own area of research is connected in some way to peroxisomes. Written by the leading experts in the field, this book surveys peroxisomal metabolic pathways, protein targeting and biogenesis of the organelle and prospects for the manipulation of peroxisomal function for biotechnological purposes. It aims to draw together the current state of the art as a convenient starting point for anyone, student or researcher, who wishes to know about plant peroxisomes.
Biomarkers of Inborn Errors in Metabolism: Clinical Aspects and Laboratory Determination is structured around the new reality that laboratory testing and biomarkers are an integral part in the diagnosis and treatment of inherited metabolic diseases. The book covers currently used biomarkers as well as markers that are in development. Because biomarkers used in the initial diagnosis of disease may be different than the follow-up markers, the book also covers biomarkers used in both the prognosis and treatment of inherited metabolic disorders. With the introduction of expanded new-born screening for inborn metabolic diseases, an increasing numbers of laboratories are involved in follow-up confirmatory testing. The book provides guidance on laboratory test selection and interpreting results in patients with suspected inherited metabolic diseases. The book provides comprehensive guidance on patient diagnosis and follow-up through its illustrative material on metabolic pathways, genetics and pathogenesis, treatment and prognosis of inherited metabolic diseases, along with essential information on clinical presentation. Each chapter is organized with a uniform, easy-to-follow format: a brief description of the disorder and pathway; a description of treatment; biomarkers for diagnosis; biomarkers followed for treatment efficacy; biomarkers followed for disease progression; confounding conditions that can either: affect biomarker expression or mimic IEMs; other biomarkers: less established, future. - Provides comprehensive information on the tests/biomarkers selection in newborn screening and follow-up of newborn screens - Categorizes biomarkers into diagnostic markers, disease follow-up markers, and prognostic biomarkers - Covers confounding factors that can alter biomarkers in the absence of inborn errors of metabolism - Offers guidance on how to distinguish acquired causes from inborn errors of metabolism
Provides an update on several new aspects of peroxisome biology, including the role of the peroxisome proliferator activated receptor. The book covers morphilogical, biochemical and molecular biological aspects of peroxisomes.
A state-of-the-art consensus report on what is known about peroxisome proliferation, the mechanisms involved, and their relevance to carcinogenesis. Peroxisomes are single, membrane-limited, cytoplasmic organelles that are found in cells of animals, plants, fungi, and protozoa. Peroxisome proliferators include certain hypolipidaemic drugs, phthalate ester plasticizers, industrial solvents, herbicides, food flavours, leukotriene D4 antagonists, and hormones. Numerous studies in rats and mice have demonstrated the hepatocarcinogenic effects of peroxisome proliferators, and these compounds have been unequivocally established as carcinogens. Since humans are exposed to peroxisome proliferators to a significant extent, assessment of the adverse biological effects of this group of compounds, and particularly their potential carcinogenicity, has become an important issue. The report has two parts. The first records the consensus reached by a group of eleven experts, including several of the leading investigators in this field. Questions addressed include the mechanisms by which peroxisome proliferators exert their carcinogenic effects in rodents, the relevance of animal studies to the evaluation of carcinogenic risk in humans, and the potential use of peroxisome proliferation as a biological marker for liver cancer. The report concludes that compounds inducing peroxisome proliferation in rats and mice have little, if any, effect on human liver. The report also issues advice on the interpretation of peroxisome proliferation, demonstrated in animal studies, when evaluating the carcinogenic risk to humans. The second part consists of three background papers presented by members of the working group.
The peroxisome is a cellular organelle which performs a vital role in animals and plants. Many advances have been made in our understanding of the peroxisome in recent years. This book provides a basic introduction to the peroxisome, followed by detailed and comprehensive discussion of its structure and function, and coverage of human peroxisomal diseases. This is an authoritative and readable text, presented in a convenient format with numerous diagrams and chapter summaries, suitable for students and researchers in the biomedical sciences.
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.
This book contains the proceedings of the International Symposium on the Mechanisms of Sexual Reproduction in Animals and Plants, where many plant and animal reproductive biologists gathered to discuss their recent progress in investigating the shared mechanisms and factors involved in sexual reproduction. This now is the first book that reviews recent progress in almost all fields of plant and animal fertilization. It was recently reported that the self-sterile mechanism of a hermaphroditic marine invertebrate (ascidian) is very similar to the self-incompatibility system in flowering plants. It was also found that a male factor expressed in the sperm cells of flowering plants is involved in gamete fusion not only of plants but also of animals and parasites. These discoveries have led to the consideration that the core mechanisms or factors involved in sexual reproduction may be shared by animals, plants and unicellular organisms. This valuable book is highly useful for reproductive biologists as well as for biological scientists outside this field in understanding the current progress of reproductive biology.