Summary

Summary Advances in Experimental Medicine and Biology 1299 Peroxisome Biology: Experimental Models, Peroxisomal Disorders and Neurological Diseases

0 purchase

Institution
123 University

Advances in Experimental Medicine and Biology provides a platform for scientific contributions in the main disciplines of the biomedicine and the life sciences. This series publishes thematic volumes on contemporary research in the areas of microbiology, immunology, neurosciences, biochemistry, ...

[Show more]

Preview 10 out of 209 pages

View example

Uploaded on October 23, 2024
Number of pages 209
Written in 2024/2025
Type Summary

advances in experimental medicine and biology 1299
peroxisome biology experimental models peroxisom

$12.99

Add to cart

Add to wishlist

100% satisfaction guarantee
Immediately available after payment
Both online and in PDF
No strings attached

Advances in Experimental Medicine and Biology 1299

Gérard Lizard Editor

Peroxisome Biology:
Experimental Models,
Peroxisomal Disorders
and Neurological
Diseases

,Advances in Experimental Medicine
and Biology

Volume 1299

Series Editors
Wim E. Crusio, Institut de Neurosciences Cognitives et Intégratives
d’Aquitaine, CNRS and University of Bordeaux, Pessac Cedex, France
Haidong Dong, Departments of Urology and Immunology, Mayo Clinic,
Rochester, MN, USA
Heinfried H. Radeke, Institute of Pharmacology & Toxicology, Clinic of the
Goethe University Frankfurt Main, Frankfurt am Main, Hessen, Germany
Nima Rezaei, Research Center for Immunodeﬁciencies, Children’s Medical
Center, Tehran University of Medical Sciences, Tehran, Iran
Junjie Xiao, Cardiac Regeneration and Ageing Lab, Institute of Cardiovascular
Sciences, School of Life Science, Shanghai University, Shanghai, China

,Advances in Experimental Medicine and Biology provides a platform for
scientiﬁc contributions in the main disciplines of the biomedicine and the life
sciences. This series publishes thematic volumes on contemporary research in
the areas of microbiology, immunology, neurosciences, biochemistry, biomed-
ical engineering, genetics, physiology, and cancer research. Covering emerging
topics and techniques in basic and clinical science, it brings together clinicians
and researchers from various ﬁelds.
Advances in Experimental Medicine and Biology has been publishing
exceptional works in the ﬁeld for over 40 years, and is indexed in SCOPUS,
Medline (PubMed), Journal Citation Reports/Science Edition, Science
Citation Index Expanded (SciSearch, Web of Science), EMBASE, BIOSIS,
Reaxys, EMBiology, the Chemical Abstracts Service (CAS), and Pathway
Studio.
2019 Impact Factor: 2.450 5 Year Impact Factor: 2.324

More information about this series at http://www.springer.com/series/5584

,Gérard Lizard
Editor

Peroxisome Biology:
Experimental Models,
Peroxisomal Disorders
and Neurological
Diseases

,Editor
Gérard Lizard
Team ‘Biochemistry of the Peroxisome, Inﬂammation and
Lipid Metabolism’ EA 7270/University of Bourgogne
Franche-Comté/Inserm, Dijon, France

ISSN 0065-2598 ISSN 2214-8019 (electronic)
Advances in Experimental Medicine and Biology
ISBN 978-3-030-60203-1 ISBN 978-3-030-60204-8 (eBook)
https://doi.org/10.1007/978-3-030-60204-8

# Springer Nature Switzerland AG 2020
This work is subject to copyright. All rights are reserved by the Publisher, whether the whole or
part of the material is concerned, speciﬁcally the rights of translation, reprinting, reuse of
illustrations, recitation, broadcasting, reproduction on microﬁlms or in any other physical way,
and transmission or information storage and retrieval, electronic adaptation, computer software, or
by similar or dissimilar methodology now known or hereafter developed.
The use of general descriptive names, registered names, trademarks, service marks, etc. in this
publication does not imply, even in the absence of a speciﬁc statement, that such names are
exempt from the relevant protective laws and regulations and therefore free for general use.
The publisher, the authors, and the editors are safe to assume that the advice and information in
this book are believed to be true and accurate at the date of publication. Neither the publisher nor
the authors or the editors give a warranty, expressed or implied, with respect to the material
contained herein or for any errors or omissions that may have been made. The publisher remains
neutral with regard to jurisdictional claims in published maps and institutional afﬁliations.

This Springer imprint is published by the registered company Springer Nature Switzerland AG.
The registered company address is: Gewerbestrasse 11, 6330 Cham, Switzerland

,This book is dedicated to my family (Sarab, Diane, Denis and
Garance) and to my collaborator Amira Z.: you are my
“lighthouses”.
I also thank my parents for having instilled in me the sense of
work, effort and perseverance without forgetting curiosity
and resourcefulness which are indispensable qualities in
research.
Louis Pasteur’s quote: “Le hasard ne favorise que les esprits
préparés”

,Preface

The discovery of the peroxisome as well as that of the lysosome is due to Prof.
Christian de Duve (1917–2013). This work earned him to share the Nobel
Prize for Medicine and Physiology in 1974 with Albert Claude and George
E. Palade. At present, the peroxisome is still a poorly known organelle, and it is
well established that peroxisomal alterations (absence of biogenesis or deﬁ-
ciency in enzymes or peroxisomal transporters) due to genetic abnormalities
are capable of inducing diseases called peroxysomopathies, some of which can
be fatal. These peroxysomopathies are often associated with developmental
disorders and anomalies of the central or peripheral nervous system. Currently,
there are no drugs to treat these diseases, and for some of them, such as
X-linked adrenoleukodystrophy (X-ALD), only allogeneic bone marrow trans-
plantation can be effective and great hopes are currently pinned on gene
therapy. A better understanding of the peroxisome, its biogenesis, its activities
and its functions is therefore an important medical issue. Recently, several
studies have suggested that the peroxisome may also be involved in more
common neurodegenerative diseases such as Alzheimer’s disease and multiple
sclerosis. These results, which deserve conﬁrmation, are in favour of the
involvement of peroxisomal dysfunction in neurodegeneration. As
neurodegeneration is often associated with oxidative stress which can generate
lipid peroxidation products, it has been shown that some of these products,
called oxysterols (formed by the auto-oxidation of cholesterol, by enzymatic
reactions or by both), induce cytotoxicity in nerve cells and lead to morpho-
logical and functional peroxisomal modiﬁcations. Today, as it is known that
peroxisomal dysfunction can favour oxidative stress and inﬂammation as well
as mitochondrial dysfunctions which are hallmarks of neurodegenerative
diseases, and that mitochondria and peroxisome are tightly connected
organelles, these arguments have reinforced the interest to study the peroxi-
some in neurodegenerative diseases other than peroxisomopathies. This book
by internationally renowned European, Canadian and Japanese researchers on
the biology and biochemistry of the peroxisome and its associated diseases is
divided into ﬁve parts: I—Biology and biochemistry of peroxisomes; II—
Peroxisomal diseases: biological characteristics and diagnosis; III—Potential
roles of peroxisomes in major neurodegenerative diseases; IV—Cell and
animal model systems; and V—Treatments of peroxisomal diseases.

vii

,viii Preface

This book provides information intended for a wide biomedical public as
well as for researchers wishing to acquire broad and recent knowledge on
peroxisome, the pathologies associated with it, their diagnoses, possible
treatments and treatment perspectives.

Dijon, France Gérard Lizard

,Contents

Part I Biology and Biochemistry of Peroxisomes
1 Peroxisome: Metabolic Functions and Biogenesis . . . . . . . . 3
Kanji Okumoto, Shigehiko Tamura, Masanori Honsho,
and Yukio Fujiki
2 Peroxisomal Dysfunction and Oxidative Stress in
Neurodegenerative Disease: A Bidirectional Crosstalk . . . . 19
Marc Fransen, Iulia Revenco, Hongli Li, Cláudio F. Costa,
Celien Lismont, and Paul P. Van Veldhoven
3 7-ketocholesterol- and 7ß-hydroxycholesterol-Induced
Peroxisomal Disorders in Glial, Microglial and Neuronal
Cells: Potential Role in Neurodegeneration . . . . . . . . . . . . 31
Thomas Nury, Aline Yammine, Franck Menetrier,
Amira Zarrouk, Anne Vejux, and Gérard Lizard
Part II Peroxisomal Diseases: Biological Characteristics
and Diagnosis
4 Peroxisome Biogenesis Disorders . . . . . . . . . . . . . . . . . . . . 45
Masanori Honsho, Kanji Okumoto, Shigehiko Tamura,
and Yukio Fujiki
5 Fatty Acid Oxidation in Peroxisomes: Enzymology,
Metabolic Crosstalk with Other Organelles and
Peroxisomal Disorders . . . . . . . . . . . . . . . . . . . . . . . . . . . . 55
Ronald J. A. Wanders, Frédéric M. Vaz, Hans R. Waterham,
and Sacha Ferdinandusse
6 Zellweger Syndrome Disorders: From Severe Neonatal
Disease to Atypical Adult Presentation . . . . . . . . . . . . . . . . 71
David Cheillan
7 Heimler Syndrome . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 81
S. Mechaussier, I. Perrault, H. Dollfus, A. Bloch-Zupan,
N. Loundon, L. Jonard, and S. Marlin

ix

, x Contents

Part III Potential Roles of Peroxisomes in Major
Neurodegenerative Diseases
8 Potential Involvement of Peroxisome in Multiple Sclerosis
and Alzheimer’s Disease . . . . . . . . . . . . . . . . . . . . . . . . . . . 91
Amira Zarrouk, Thomas Nury, Hammam I. El Hajj,
Catherine Gondcaille, Pierre Andreoletti, Thibault Moreau,
Mustapha Cherkaoui-Malki, Johannes Berger,
Mohamed Hammami, Gérard Lizard, and Anne Vejux
9 Developmental and Degenerative Cerebellar Pathologies
in Peroxisomal β-Oxidation Deﬁciency . . . . . . . . . . . . . . . . 105
Stephanie De Munter and Myriam Baes
Part IV Cell and Animal Model Systems
10 A Mouse Model System to Study Peroxisomal Roles in
Neurodegeneration of Peroxisome Biogenesis Disorders . . . 119
Yuichi Abe, Shigehiko Tamura, Masanori Honsho,
and Yukio Fujiki
11 The Drosophila melanogaster as Genetic Model System to
Dissect the Mechanisms of Disease that Lead to
Neurodegeneration in Adrenoleukodystrophy . . . . . . . . . . 145
Margret H. Bülow, Brendon D. Parsons, and Francesca Di Cara
12 Human Peroxisomal 3-Ketoacyl-CoA Thiolase: Tissue
Expression and Metabolic Regulation . . . . . . . . . . . . . . . . 161
Norbert Latruffe
Part V Treatments of Peroxisomal Diseases
13 Biological Functions of Plasmalogens . . . . . . . . . . . . . . . . . 171
Md Shamim Hossain, Shiro Mawatari, and Takehiko Fujino
14 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 . . . . . . . . . . . . . . . . . . . . . 195
Takehiko Fujino, Md Shamim Hossain, and Shiro Mawatari

The benefits of buying summaries with Stuvia:

Guaranteed quality through customer reviews

Stuvia customers have reviewed more than 700,000 summaries. This how you know that you are buying the best documents.

Quick and easy check-out

You can quickly pay through credit card or Stuvia-credit for the summaries. There is no membership needed.

Focus on what matters

Your fellow students write the study notes themselves, which is why the documents are always reliable and up-to-date. This ensures you quickly get to the core!

Frequently asked questions

What do I get when I buy this document?

You get a PDF, available immediately after your purchase. The purchased document is accessible anytime, anywhere and indefinitely through your profile.

Satisfaction guarantee: how does it work?

Our satisfaction guarantee ensures that you always find a study document that suits you well. You fill out a form, and our customer service team takes care of the rest.

Who am I buying these notes from?

Stuvia is a marketplace, so you are not buying this document from us, but from seller Exammate. Stuvia facilitates payment to the seller.

Will I be stuck with a subscription?

No, you only buy these notes for $12.99. You're not tied to anything after your purchase.

Can Stuvia be trusted?

4.6 stars on Google & Trustpilot (+1000 reviews)

69052 documents were sold in the last 30 days

Founded in 2010, the go-to place to buy study notes for 15 years now

Start selling