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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.
Preface
Biology and Biochemistry of Peroxisomes
Peroxisome: Metabolic Functions and Biogenesis
Peroxisomal Dysfunction and Oxidative Stress in Neurodegenerative Disease: A Bidirectional Crosstalk
7-Ketocholesterol- and 7β-Hydroxycholesterol-Induced Peroxisomal Disorders in Glial, Microglial and Neuronal Cells:...
Peroxisomal Diseases: Biological Characteristics and Diagnosis
Peroxisome Biogenesis Disorders
Fatty Acid Oxidation in Peroxisomes: Enzymology, Metabolic Crosstalk with Other Organelles and Peroxisomal Disorders
Zellweger Syndrome Disorders: From Severe Neonatal Disease to Atypical Adult Presentation
Heimler Syndrome
Potential Roles of Peroxisomes in Major Neurodegenerative Diseases
Potential Involvement of Peroxisome in Multiple Sclerosis and Alzheimer´s Disease
Developmental and Degenerative Cerebellar Pathologies in Peroxisomal β-Oxidation Deficiency
Cell and Animal Model Systems
A Mouse Model System to Study Peroxisomal Roles in Neurodegeneration of Peroxisome Biogenesis Disorders
The Drosophila melanogaster as Genetic Model System to Dissect the Mechanisms of Disease that Lead to Neurodegener...
Human Peroxisomal 3-Ketoacyl-CoA Thiolase: Tissue Expression and Metabolic Regulation
Treatments of Peroxisomal Diseases
Biological Functions of Plasmalogens
Therapeutic Efficacy of Plasmalogens for Alzheimer´s Disease, Mild Cognitive Impairment, and Parkinson´s Disease i