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.

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.

Lipobiology
Lipobiology

Author: G. J. van der Vusse

Publisher: Gulf Professional Publishing

Published: 2004

Total Pages: 640

ISBN-13: 9780444514967

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

Author: Gérard Lizard

Publisher: Springer Nature

Published: 2021-01-08

Total Pages: 209

ISBN-13: 3030602044

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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.

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.

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

Peroxisomes and their Key Role in Cellular Signaling and Metabolism
Peroxisomes and their Key Role in Cellular Signaling and Metabolism

Author: Luis A. del Río

Publisher: Springer Science & Business Media

Published: 2013-07-03

Total Pages: 353

ISBN-13: 9400768893

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Peroxisomes are a class of ubiquitous and dynamic single membrane-bounded cell organelles, devoid of DNA, with an essentially oxidative type of metabolism. In recent years it has become increasingly clear that peroxisomes are involved in a range of important cellular functions in almost all eukaryotic cells. In higher eukaryotes, including humans, peroxisomes catalyze ether phospholipids biosynthesis, fatty acid alpha-oxidation, glyoxylate detoxification, etc, and in humans peroxisomes are associated with several important genetic diseases. In plants, peroxisomes carry out the fatty acid beta-oxidation, photorespiration, metabolism of ROS, RNS and RSS, photomorphogenesis, biosynthesis of phytohormones, senescence, and defence against pathogens and herbivores. In recent years it has been postulated a possible contribution of peroxisomes to cellular signaling. In this volume an updated view of the capacity and function of peroxisomes from human, animal, fungal and plant origin as cell generators of different signal molecules involved in distinct processes of high physiological importance is presented.