Main description:

Mitochondria have long been the Rodney Dangerfield of cellular organelles. Believed to be the remnants of bacterial infection of eukaryotic cells eons ago, the mitochondrion evolved a symbiotic relationship in which it dutifully served as the efficient source of A TP for cell function. The extraordinary dependence of cells on the energy provided by mito chondrial oxidative metabolism of glucose, especially through critical organs such as the heart and brain, is underlined by the fatal consequences of toxins that interfere with the mitochondrial electron transport system. Consistent with their ancestry, the mitochondria have their own DNA that encodes many but not all of their proteins. The mitochon dria and their genes come from the mother via the ovum since sperm do not possess mitochondria. This extranuclear form of inheritance derived exclusively from the female side has proven to be a powerful tool for tracing the evolution by the number of base substitutions in mtDNA. That mitochondrial gene mutations might be a source of human dis ease became evident a decade ago with the characterization of a group of multisystem disorders, typically involving the nervous system, which are transmitted from mother to child. Specific point mutations in mtDNA have been associated with the different syndromes.

Contents:

Part I. Mitochondrial Toxins: Symptomatology, Origin, and Chemistry. Clinical Manifestations and Mechanisms of Action of Environmental Mitochondrial Toxins, Mohammad I. Sabri, Peter S. Spencer, Safia Baggia, and Albert C. Ludolph. History of 3-Nitropropionic Acid: Occurrence and Role in Human and Animal Disease, Bradley F. Hamilton, Daniel H. Gould, and David L. Gustine. The Neurochemistry of 3-Nitropropionic Acid, Norman C. Reynolds, Jr. and Wen Lin. Part II. Mitochondrial Dysfunctions: Models of Neurodegeneration and Mechanisms of Action. In Vitro Studies of 3-Nitropropionic Acid, Gail D. Zeevalk. Cognitive and Motor Deficits Produced by Acute and Chronic Administration of 3-Nitropropionic Acid in Rats, Gary L. Dunbar, Deborah A. Shear, Jie Dong, and Kristi L. Haik-Creguer. Comparative Study on 3-Nitropropionic Acid Neurotoxicity, Cesario V. Borlongan. Mechanisms of 3-Nitropropionic Acid Neurotoxicity, James W. Geddes, Vimala Bondada, and Zhen Pang. Gender-Related Difference of the Effect of 3-Nitropropionic Acid on Striatal Artery, Keiya Nakajima, Yasunobu Shimano, Kunio Torii, and Hitoo Nishino. Variable Susceptibility to Neurotoxicity of Systemic 3-Nitropropionic Acid, Tajrena Alexi, Richard L. M. Faull, and Paul E. Hughes. The 3-Nitropropionic Acid Model of Huntington's Disease: Do Alterations in the Expression of Metabolic mRNAs Predict the Development of Striatal Pathology? Keith J. Page, Alicia Meldrum, and Stephen B. Dunnett. Mechanisms of Action of 3-Nitropropionic Acid: Dopamine Overflow and Vulnerability of the Lateral Striatal Artery, Michiko Kumazaki, Chucharin Ungsuparkorn, Shripad B. Deshpande, Atsuo Fukuda, and Hitoo Nishino. Mitochondrial Inhibition and Neuronal Death in Huntington's Disease, Maria Isabel Behrens. Effects of Brain Mitochondrial Metabolism, Aging, and Caloric Restriction on Membrance Lipids and Proteins: An Electron Paramagnetic Resonance Investigation, S. Prasad Gabbita, John M. Carney, and D. Allan Butterfield. Malonate: Profileand Mechanisms of Striatal Toxicity, Alicia Meldrum, Keith J. Page, Barry J. Everitt, and Stephen B. Dunnett. Malonic Acid and the Chronic Administration Model of Excitotoxicity, Terence J. Bazzett, Roger L. Albin, and Jill B. Becker. Sodium Azide-Induced Neurotoxicity, Yun Wang and Cesario V. Borlongan. Part III. Treatment Interventions for Mitochondrial-Induced Neurotoxicity. Neuroprotective Strategies Against Cellular Hypoxia, Matthias W. Riepe. Neuroprotective Effect of Perinatal Hypoxia Against 3-Nitropropionic Acid Neurotoxicity, Zbigniew K. Binienda and Andrew C. Scallet. Neural Transplantation and Huntington's Disease: What Can We Learn from the 3-Nitropropionic Acid Model? Cesario V. Borlongan, Christine E. Stahl, Thomas B. Freeman, Robert A. Hauser, and Paul R. Sanberg. Neuroprotective Strategies in Parkinson's Disease and Huntington's Chorea: MPTP- and 3-NPA-Induced Neurodegeneration as Models, Moussa B. H. Youdim, Gopal Krishna, and Chuang C. Chiueh. Index.