Handbook of Mitochondrial Dysfunction

Mitochondria produce the chemical energy necessary for eukaryotic cell functions; hence mitochondria are an essential component of health, playing roles in both disease and aging. More than 80 human diseases and syndromes are associated with mitochondrial dysfunction; this book focuses upon diseases linked to these ubiquitous organelles. Accumulation of mitochondrial DNA damage results in mitochondrial dysfunction through two main pathways. Mutation in mitochondrial DNA causes diseases such as Kearns-Sayre syndrome and Pearson syndrome. Mutation in chromosomal DNA causes diseases such as Parkinson's disease and schizophrenia. These and many other diseases are reviewed in this book.

Key Features

• Presents the detailed structure of mitochondria, mitochondrial function, roles of oxidants and antioxidants in mitochondrial dysfunction.
• Includes summary of both causes and effects of these diseases.
• Discusses current and potential future therapies for mitochondrial dysfunction diseases
• Explores a wide variety of diseases caused by dysfunctional mitochondria.

Contents

Preface ........................................................................................................................................................................... xi

Acknowledgment......................................................................................................................................................... xiii

About the Editor .......................................................................................................................................................... xv

Contributors ............................................................................................................................................................... xvii

SECTION I Introduction: Structure and Function of Mitochondria

Chapter 1 Mitochondrial Structure and Function ....................................................................................................... 3

Marcos Roberto de Oliveira

Chapter 2 Structure, Function and Evolutionary Aspects of Mitochondria ................................................................ 9

Puja Agarwal, Mehali Mitra, and Sujit Roy

Chapter 3 Mitochondrial Genome Damage, Dysfunction and Repair ........................................................................23

Kalyan Mahapatra, Sayanti De, and Sujit Roy

SECTION II Mitochondrial Dysfunction and Diseases

Chapter 4 Instability of Human Mitochondrial DNA, Nuclear Genes and Diseases ..................................................39

Katarzyna Tońska

Chapter 5 Kearns-Sayre Syndrome .............................................................................................................................55

Josef Finsterer

Chapter 6 Mitochondrial Dysfunction and Barth Syndrome......................................................................................61

Arianna F. Anzmann, Steven M. Claypool, and Hilary Vernon

Chapter 7 Mitochondrial Dysfunction in the Pathophysiology of Alzheimer’s Disease ..............................................79

Isaac G. Onyango

Chapter 8 Mitochondrial Dysfunction in Multiple Sclerosis.......................................................................................87

Angela Dziedzic, Elzbieta Miller, Joanna Saluk-Bijak, and Michał Bijak

Chapter 9 Mitochondrial Dysfunction in Chronic Kidney Disease.............................................................................93

Maria V. Irazabal and Alfonso Eirin

Chapter 10 Mitochondrial Dysfunction in Breast Cancer: A Potential Target from the Bench to Clinical

Therapeutics.............................................................................................................................................103

Thalita Basso Scandolara, Letícia Madureira Pacholak, Thayse Fachin Cormanique, Rodrigo Kern,

and Carolina Panis

Chapter 11 Mitochondrial Dysfunction and DNA Methylation in Atherosclerosis ....................................................115

Silvio Zaina and Gertrud Lund

Chapter 12 Mitochondrial Dysfunction and Heart Diseases ......................................................................................127

Dao-Fu Dai

Chapter 13 The Role of Mitochondrial Dysfunction in Mitophagy and Apoptosis and Its Relevance to Cancer .......143

Jan Ježek

Chapter 14 Mitochondrial Dysfunction Linking Obesity and Asthma .......................................................................151

Naveen K. Bhatraju and Anurag Agrawal

Chapter 15 Mitochondrial Dysfunction and Hearing Loss.........................................................................................163

María de la Luz Arenas Sordo

Chapter 16 Superoxide Dismutase, Mitochondrial Dysfunction, and Neurodegenerative Diseases ............................171

Jahaun Azadmanesh and Gloria E. O. Borgstahl

Chapter 17 Role of Post-Translational Modifications of Mitochondrial Complex I in Mitochondrial Dysfunction

and Human Brain Pathologies .................................................................................................................191

Vismaya, Yogachar Chithra, and Muchukunte Mukunda Srinivas Bharath

Chapter 18 Heme Oxygenase-1 in Kidney Health and Disease: Perspective of Mitochondrial Homeostasis ..............205

Pu Duann, Elias A. Lianos, and Pei-Hui Lin

Chapter 19 Mitochondrial Transplantation in Myocardial Ischemia and Reperfusion Injury ....................................217

David Blitzer, Borami Shin, Alvise Guariento, Pedro J. del Nido, and James D. McCully

Chapter 20 Mitochondrial Dysfunction in Diabetes ...................................................................................................231

Eric L. Johnson

Chapter 21 Clinical Manifestation of Mitochondrial Disorders in Childhood............................................................243

Martin Magner

Chapter 22 Mitochondrial Dysfunction and Epilepsies ..............................................................................................265

Bindu Parayil Sankaran and Arun B. Taly

Chapter 23 Mitochondrial Dysfunction and Allergic Disease.....................................................................................277

Kritika Khanna and Anurag Agrawal

SECTION III Factors Affecting Mitochondrial Function

Chapter 24 Role of Carnosic Acid in Protection of Brain Mitochondria ....................................................................293

Marcos Roberto de Oliveira

Chapter 25 Mitochondrial Dysfunction and Its Impact on Oxidative Capacity of Muscle .........................................309

Teet Seene

Chapter 26 The Effects of Resveratrol on the Brain Mitochondria.............................................................................319

Marcos Roberto de Oliveira

Chapter 27 Pathophysiology and Biomarkers of Mitochondrial Dysfunction Induced by Heavy Exercise .................335

Sergej M. Ostojic

Chapter 28 Mitochondrial Pathologies and Their Neuromuscular Manifestations .....................................................341

Carlos Ortez and Andrés Nascimento

Chapter 29 Mitochondrial Stress and Cellular Senescence..........................................................................................361

Irene L. Tan and Michael C. Velarde

Chapter 30 Role of Mitochondrial Dysfunction in Human Obesity ...........................................................................379

David Albuquerque, Sara Silva, Daniel Álvarez-Vaca, Célia Aveleira, and Clévio Nóbrega

Chapter 31 Roles of Melatonin in Maintaining Mitochondrial Welfare: Focus on Renal Cells...................................389

Feres José Mocayar Marón, Emiliano Diez, Russel J. Reiter, and Walter Manucha

Chapter 32 Mitochondrial Dysfunction Affecting the Peripheral Nervous System in Diabetic Neuropathy

and Avenues for Therapy..........................................................................................................................399

Jennifer Jossart, Taylor N. Dennis, and J. Jefferson P. Perry

Chapter 33 Mitochondrial Dysfunction and Oxidative Stress in the Pathogenesis of Metabolic Syndrome................409

Qiaozhu Su and Hao Wang

SECTION IV Immunity and Toxicity

Chapter 34 Regulation of Antiviral Immunity by Mitochondrial Dynamics ..............................................................425

Mohsin Khan, Hasan Imam, and Saiful Anam Mir

Chapter 35 Mitochondrial Dysfunction, Immune Systems, Their Diseases, and Possible Treatments.........................443

Elise Jacquin, Eric Hervouet, and Michaël Boyer-Guittaut

Chapter 36 Organophosphorus Compound-Induced Mitochondrial Disruption........................................................461

Alan J. Hargreaves

Chapter 37 Mitochondrial Dysfunction in Friedreich Ataxia .....................................................................................471

Rosella Abeti, Annalisa Baccaro, and Paola Giunti

Index............................................................................................................................................................................477

Shamim I. Ahmad earned his

Master’s degree in Botany from

Patna University, Bihar, India, and

his PhD in Molecular Genetics

from Leicester University, England.

He then joined Nottingham

Polytechnic as a grade 1 lecturer and subsequently was

promoted to the post of Senior Lecturer. Ahmad now

spends much of his time producing/writing medical

books. For more than three decades he researched different

areas of molecular biology/genetics including thymineless

death in bacteria, genetic control of nucleotide

catabolism, development of anti-AIDs drug, control of

microbial infection of burns, phages of thermophilic bacteria,

and microbial flora of Chernobyl after the nuclear

power station accident. But his main interest is DNA

damage and repair specifically by near-ultraviolet light

mostly through the photolysis of biological compounds,

production of reactive oxygen species, and their implications

on human health including skin cancer. He also

investigates near-ultraviolet photolysis of non-biological

compounds such as 8-metoxypsoralen, mitomycin C, and

their importance in psoriasis treatment and in Fanconi

anemia. He has collaborated with the University of

Osaka, Japan, and he and his colleagues were able to

show that a number of naturally occurring enzymes were

able to scavenge the reactive oxygen species.

In 2003 he received a prestigious Asian Jewel Award

in Britain for Excellence in Education. His long time ambition

to produce medical books started in 2007 and since

then he has published Molecular mechanisms of Fanconi

Anemia, Molecular Mechanisms of Xeroderma Pigmentosum;

Molecular Mechanisms of Cockayne Syndrome;

Molecular Mechanisms of Ataxia Telangiectasia; Diseases

of DNA repair; Neurod