Exploring Mitochondria in Neurodegenerative Diseases

in Neurodegenerative Diseases contains an impressive overview of the role of mitochon-drial dysfunction as the primary effector of, or relevant mechanism leading to, neurodegeneration. In the 26 Mitochondria and Free Radicals in Neurodegenerative chapters featured in this book, the authors guide the Diseases reader from the basic concepts of mitochondrial function and free radical generation to the role of mitochon-New York: Wiley-Liss (1997). 610 pp., $99.95. drial defects in disease. Topics covered include the mechanism of mitochondrial dysfunction in excitotox-icity, the role of mitochondria as a source of oxidative stress in apoptosis and neurodegeneration, the role of Mitochondria became an integral part of eukaryotic cells mitochondrial DNA mutations in the onset of disease, as a result of a mutually advantageous bargain between and possible therapeutic approaches. The majority of an archaic opportunistic bacterium and a host cell. The the chapters gathered by the authors are informative terms of this agreement included a dangerous clause: and very well written, and provide a high quality presen-should the cell attempt to eliminate the mitochondrion, tation of the current knowledge regarding the impair-or should the latter cease to supply ATP, the result would ment of mitochondrial function in neurodegenerative be reciprocal elimination. Furthermore, it is now clear conditions. The implications of mitochondrial DNA mu-that in addition to generating energy for cell function tations for the onset of neurodegeneration are exhaus-and survival, mitochondria also store factors that, when tively described in several chapters; less emphasized released, can cause cell death. To prevent their elimina-are mechanisms by which mutations in nonmitochon-tion during evolution, these factors are obviously also drial genes may lead to mitochondrial dysfunction. involved in maintenance of mitochondrial function and One of the general themes of the book revolves around cell survival. the energy-linked excitotoxicity hypothesis, which sug-Mitochondrial dysfunction may indeed be involved in gests that a primary energy failure would lead to partial the development of neurodegenerative diseases such as Huntington's, Parkinson's, and Alzheimer's diseases and ischemic brain damage. Genetic mitochondrial defects are also a probable primary pathogenic cause of some neurodegenerative conditions. Of the ‫0031ف‬ genes that the archaic bacteria carried into the host cell, only 13 protein-coding genes have been retained by mitochondria, supported by 22 tRNA and 2 rRNA genes. Mitochondrial DNA has unique characteristics. It is believed to be maternally inherited, and mutations create a heterogeneous population of normal and altered molecules that are distributed randomly in daughter cells. …