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Title Peroxisome biology : experimental models, peroxisomal disorders and neurological diseases / Gérard Lizard, editor
Published Cham : Springer, 2020


Description 1 online resource
Series Advances in Experimental Medicine and Biology, 0065-2598 ; 1299
Advances in experimental medicine and biology ; v. 1299.
Contents [II]. Peroxisomal diseases : biological characteristics and diagnosis: Peroxisome biogenesis disorders / Masanori Honsho, Kanji Okumoto, Shigehiko Tamura, Yukio Fujiki -- Fatty acid oxidation in peroxisomes : enzymology, metabolic crosstalk with other organelles and peroxisomal disorders / Ronald J. A. Wanders, Frédéric M. Vaz, Hans R. Waterham, Sacha Ferdinandusse -- Zellweger syndrome disorders : from severe neonatal disease to atypical adult presentation / David Cheillan -- Heimler syndrome / S. Mechaussier, I. Perrault, H. Dollfus, A. Bloch-Zupan, N. Loundon, L. Jonard et al
[IV]. Cell and animal model systems: A mouse model system to study peroxisomal roles in neurodegeneration of peroxisome biogenesis disorders / Yuichi Abe, Shigehiko Tamura, Masanori Honsho, Yukio Fujiki -- The Drosophila melanogaster as genetic model system to dissect the mechanisms of disease that lead to neurodegeneration in adrenoleukodystrophy / Margret H. Bülow, Brendon D. Parsons, Francesca Di Cara -- Human peroxisomal 3-ketoacyl-CoA thiolase : tissue expression and metabolic regulation / Norbert Latruffe
[V]. Treatment of peroxisomal diseases: Biological functions of plasmalogens / Md Shamim Hossain, Shiro Mawatari, Takehiko Fujino -- Therapeutic efficacy of plasmalogens for Alzheimer's disease, mild cognitive impairment, and Parkinson's disease in conjunction with a new hypothesis for the etiology of Alzheimer's disease / Takehiko Fujino, Md Shamim Hossain, Shiro Mawatari
Summary 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
Bibliography Includes bibliographical references
Subject Peroxisomes.
Peroxisomal Disorders -- genetics
Form Electronic book
Author Lizard, Gérard, editor
ISBN 9783030602048