Lipobiology
Lipobiology

Author: Ger J. van der Vusse

Publisher: Gulf Professional Publishing

Published: 2004

Total Pages: 640

ISBN-13: 9780444514967

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Molecular Machines Involved in Peroxisome Biogenesis and Maintenance
Molecular Machines Involved in Peroxisome Biogenesis and Maintenance

Author: Cecile Brocard

Publisher: Springer

Published: 2014-07-23

Total Pages: 540

ISBN-13: 3709117887

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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.

Peroxisome Biology: Experimental Models, Peroxisomal Disorders and Neurological Diseases
Peroxisome Biology: Experimental Models, Peroxisomal Disorders and Neurological Diseases

Author: Gérard Lizard

Publisher: Springer

Published: 2021-01-09

Total Pages: 212

ISBN-13: 9783030602031

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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.

Peroxisomes: Biogenesis, Function, and Role in Human Disease
Peroxisomes: Biogenesis, Function, and Role in Human Disease

Author: Tsuneo Imanaka

Publisher: Springer Nature

Published: 2020-01-16

Total Pages: 284

ISBN-13: 9811511691

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This book provides readers with a comprehensive overview of peroxisomes and their role in human diseases. It starts by describing the history of peroxisome research and then examines in detail the current understanding of the biogenesis and function of peroxisomes. It then focuses on peroxisomal disorders and the involvement of peroxisomes in cancer and age-related diseases, discussing in detail the use of model organisms to elucidate the pathogenesis of peroxisomal disorders and the physiological importance of peroxisomal proteins. Further, the book examines diagnostic and therapeutic strategies in peroxisomal disorders as well as significant recent advances. Lastly, it addresses various topics in peroxisome research, including the isolation of peroxisomes from mammalian tissues and cells, the structural biology of peroxisomal proteins, the lipidomics of peroxisomal disorders, the value of exome sequencing, and neuropsychological testing in X-linked adrenoleukodystrophy. Given its scope, the book is a valuable resource for postgraduate students and researchers in the life sciences and clinicians in the fields of internal medicine, pediatrics, and neurology.

Plant Peroxisomes
Plant Peroxisomes

Author: A. Baker

Publisher: Springer

Published: 2013-01-08

Total Pages: 505

ISBN-13: 9789401598590

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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 in Inborn Errors of Metabolism
Biomarkers in Inborn Errors of Metabolism

Author: Uttam Garg

Publisher: Elsevier

Published: 2017-06-07

Total Pages: 477

ISBN-13: 0128029188

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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

The Liver
The Liver

Author: Irwin M. Arias

Publisher: John Wiley & Sons

Published: 2020-03-09

Total Pages: 1156

ISBN-13: 1119436826

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Bridging the gap between basic scientific advances and the understanding of liver disease — the extensively revised new edition of the premier text in the field. The latest edition of The Liver: Biology and Pathobiology remains a definitive volume in the field of hepatology, relating advances in biomedical sciences and engineering to understanding of liver structure, function, and disease pathology and treatment. Contributions from leading researchers examine the cell biology of the liver, the pathobiology of liver disease, the liver’s growth, regeneration, metabolic functions, and more. Now in its sixth edition, this classic text has been exhaustively revised to reflect new discoveries in biology and their influence on diagnosing, managing, and preventing liver disease. Seventy new chapters — including substantial original sections on liver cancer and groundbreaking advances that will have significant impact on hepatology — provide comprehensive, fully up-to-date coverage of both the current state and future direction of hepatology. Topics include liver RNA structure and function, gene editing, single-cell and single-molecule genomic analyses, the molecular biology of hepatitis, drug interactions and engineered drug design, and liver disease mechanisms and therapies. Edited by globally-recognized experts in the field, this authoritative volume: Relates molecular physiology to understanding disease pathology and treatment Links the science and pathology of the liver to practical clinical applications Features 16 new “Horizons” chapters that explore new and emerging science and technology Includes plentiful full-color illustrations and figures The Liver: Biology and Pathobiology, Sixth Edition is an indispensable resource for practicing and trainee hepatologists, gastroenterologists, hepatobiliary and liver transplant surgeons, and researchers and scientists in areas including hepatology, cell and molecular biology, virology, and drug metabolism.