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Subject Area(s):  Cell BiologyMolecular BiologyBiochemistry

Edited by Douglas C. Wallace, Children's Hospital of Philadelphia, University of Pennsylvania; Richard J. Youle, National Institutes of Health

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© 2014 • 270 pp., illus. (29 4C, 9 B&W), index
Hardcover • $135 94.50
ISBN  978-1-936113-35-4
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Mitochondria are subcellular organelles that function as “power plants” for the cell, generating energy in the form of ATP from glucose, oxygen, and other molecules. Thought to have arisen about 2 billion years ago when an aerobic bacterium invaded the primitive eukaryotic cell, they have their own DNA, undergo fission and fusion independently, and play an important role in programmed cell death.

Written and edited by experts in the field, this collection from Cold Spring Harbor Perspectives in Biology discusses the evolution of mitochondria, their functions in cells, and the numerous diseases in which mitochondrial dysfunction is implicated. The contributors also examine mitochondrial biogenesis, the molecular mechanisms underlying fission and fusion, how proteins are imported from the cytoplasm, and the organization of the mitochondrial DNA.

This book includes chapters covering the involvement of mitochondria in Parkinson’s disease, encephalopathies, tumorigenesis, muscular dystrophy, and other diseases, as well as aging. It is thus a vital reference for all cell and molecular biologists, as well as researchers working on muscle and neurodegenerative diseases, the role of metabolism in aging, and cancer.


Mitochondrial Evolution
Michael W. Gray
Mitochondrial DNA Genetics and the Heteroplasmy Conundrum in Evolution and Disease
Douglas C. Wallace and Dimitra Chalkia
Mechanisms of Protein Sorting in Mitochondria
Diana Stojanovski, Maria Bohnert, Nikolaus Pfanner, and Martin van der Laan
The Mitochondrial Nucleoid: Integrating Mitochondrial DNA into Cellular Homeostasis
Robert Gilkerson, Liliana Bravo, Iraselia Garcia, Norma Gaytan, Alan Herrera, Alicia Maldonado, and Brandi Quintanilla
Clinical and Molecular Features of POLG-Related Mitochondrial Disease
Jeffrey D. Stumpf, Russell P. Saneto, and William C. Copeland
Mitochondrial Biogenesis through Activation of Nuclear Signaling Proteins
John E. Dominy and Pere Puigserver
Mechanisms of Mitochondrial Fission and Fusion
Alexander M. van der Bliek, Qinfang Shen, and Sumihiro Kawajiri
Mitochondrial Quality Control Mediated by PINK1 and Parkin: Links to Parkinsonism
Derek Narendra, John E. Walker, and Richard Youle
Mitochondrial Trafficking in Neurons
Thomas L. Schwarz
Where Killers Meet—Permeabilization of the Outer Mitochondrial Membrane during Apoptosis
Tom Bender and Jean-Claude Martinou
The Role of Mitochondria in Cellular Iron–Sulfur Protein Biogenesis: Mechanisms, Connected Processes, and Diseases
Oliver Stehling and Roland Lill
Altered Sulfide (H2S) Metabolism in Ethylmalonic Encephalopathy
Valeria Tiranti and Massimo Zeviani
Mitochondrial Dysfunction and Defective Autophagy in the Pathogenesis of Collagen VI Muscular Dystrophies
Paolo Bernardi and Paolo Bonaldo
Relevance of Mitochondrial Genetics and Metabolism in Cancer Development
Giuseppe Gasparre, Anna Maria Porcelli, Giorgio Lenaz, and Giovanni Romeo
Mitochondrial Metabolism, Sirtuins, and Aging
Michael N. Sack and Toren Finkel