Mitochondria in Health and in Sickness

1. Front Matter

   Pages i-vi

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2. Mitochondrial Metabolism in Cancer. A Tangled Topic. Which Role for Proteomics?

   • Patrizia Bottoni, Roberto Scatena

   Pages 1-16

3. Mitochondrial Proteins in the Development of Parkinson’s Disease

   • Mara Zilocchi, Mauro Fasano, Tiziana Alberio

   Pages 17-44

4. Role of Mitochondria in Host-Pathogen Interaction

   • Alessio Soggiu, Paola Roncada, Luigi Bonizzi, Cristian Piras

   Pages 45-57

5. Mitochondria Lysine Acetylation and Phenotypic Control

   • Federica Ciregia

   Pages 59-70

6. Crosstalk Between Oxidative Stress and Mitochondrial Damage: Focus on Amyotrophic Lateral Sclerosis

   • Viviana Greco, Patrizia Longone, Alida Spalloni, Luisa Pieroni, Andrea Urbani

   Pages 71-82

7. A Tag-Based Affinity Purification Mass Spectrometry Workflow for Systematic Isolation of the Human Mitochondrial Protein Complexes

   • Zhuoran Wu, Ramy Malty, Mohamed Taha Moutaoufik, Qingzhou Zhang, Matthew Jessulat, Mohan Babu

   Pages 83-100

8. Targeted Proteomics to Study Mitochondrial Biology

   • Justina C. Wolters, Hjalmar P. Permentier, Barbara M. Bakker, Rainer Bischoff

   Pages 101-117

9. Recent Advances in Targeting Human Mitochondrial AAA+ Proteases to Develop Novel Cancer Therapeutics

   • Keith S. Wong, Walid A. Houry

   Pages 119-142

10. Investigating the Role of Mitochondria in Type 2 Diabetes – Lessons from Lipidomics and Proteomics Studies of Skeletal Muscle and Liver

    • Lisa Kappler, Laxmikanth Kollipara, Rainer Lehmann, Albert Sickmann

    Pages 143-182

11. Quantification of Mitochondrial Network Characteristics in Health and Disease

    • Andrew J. Valente, Joao Fonseca, Fereshteh Moradi, Gregory Foran, Alexander Necakov, Jeffrey A. Stuart

    Pages 183-196

12. Cysteine Switches and the Regulation of Mitochondrial Bioenergetics and ROS Production

    • Ryan J. Mailloux

    Pages 197-216

13. Advances Towards Therapeutic Approaches for mtDNA Disease

    • Iman Al Khatib, Timothy E. Shutt

    Pages 217-246

14. Mitochondrial Genetics

    • Vanessa F. Gonçalves

    Pages 247-255

15. Heteroplasmy Shifting as Therapy for Mitochondrial Disorders

    • Mansur M. Naeem, Neal Sondheimer

    Pages 257-267

16. Application of CRISPR-Cas9 Screening Technologies to Study Mitochondrial Biology in Healthy and Disease States

    • David Sharon, Steven M. Chan

    Pages 269-277

Besides bringing together researchers and clinicians from various disciplines to share their scope of research on the seminal role of mitochondria in human disease, this original volume of the book has a broader appeal by exploring the health and disease of mitochondria, with specific emphasis on how mitochondrial dysfunction contributes to the development of various neurodegeneration, cancer, and metabolic disorders. The book also provides a foundational overview of the mitochondrial pathogenic or genetic variants and highlights various analytical tools used in the field of mitochondrial genetics; mitochondrial replacement therapy and strategies geared towards shifting heteroplasmy in individuals with mitochondrial disease; how state-of-the-art omics technologies (proteomics, functional genomics) have been employed to study mitochondrial biology in healthy and disease states; post-translational modifications in the regulation of mitochondrial proteins; and the role of mitochondriain host-pathogen interactions. 

Current approaches taken to study steady-state characteristics of mitochondrial structure and function in live mammalian cells in the contexts of normal and diseased states, and most recent research efforts to develop compounds with anti-cancer potential by targeting mitochondrial proteases or advances in therapeutic approaches towards mitochondrial disease were also explored. By covering this broad range of topics, our hope is to disseminate a wealth of knowledge on the critical role of mitochondria, and how to probe its function in health and in sickness.

• Mitochondrial disease
• Mitochondrial dysfunction
• Mitochondrial health
• Mitochondrial metabolism
• Mitochondrial proteomics

• Institute of Biochemistry and Clinical Biochemistry, Università Cattolica del Sacro Cuore, Rome, Italy, Fondazione Policlinico Universitario A. Gemelli – IRCCS, Rome, Italy

  Andrea Urbani

• Department of Biochemistry, University of Regina, Regina, Canada

  Mohan Babu

Prof. Andrea Urbani has been involved in structural and functional investigation of integral membrane protein complexes of the respiratory chains since he moved in 1998 to Heidelberg (Germany) at the European Molecular Biology Laboratory (EMBL) in the Structural Biology Program. Mass spectrometry characterisation and identification of proteins and metabolites represents the primary motive of the laboratories he has established during the last fifteen years. He is currently the Director of the Biochemistry and Clinical Biochemistry Institute of the Catholic University of the Sacred Heart, and President of the European Proteomics Association (EuPA) for the years 2015-2019.  
Prof. Mohan Babu is internationally recognized as an emerging leader in network biology, and a pioneer for mapping epistatic relationships and membrane-associated protein complexes in prokaryotes. His multidisciplinary research program focuses on characterizing protein and genetic interaction networks from microbes to mammals, and establishing new molecular mechanisms for mitochondrial proteins associated with neurodegenerative disorders. He is an editorial board member of the journal “BioEssays”, editor of the book “Prokaryotic Systems Biology” (Springer), Associate editor of “BMC Microbiology”, secretary treasurer for “Canadian Society of Microbiology”, and an advisory board member of the “International Mitochondrial Human Proteome Initiative”.

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