Mitochondrial Disorders : From Pathophysiology to Acquired Defects

G. SERRATRICE This book is new and original. For some decades mitochondriopathies have been extensively studied: morphology, biochemistry, genetics. However these studies mainly concerned encephalopathies and myopathies related to mito- chondrial disorders. In this book the field of mitochondrial cytopathies is largely extended to "Mitochondrial Medicine". Very new data are gathered. A new step is jumped. Many medical specialities are concerned. Part I is devoted to mitochondrial biology: the mitochondrial machinery, the dysfunction of mitochondrial in elderly: cell decline, deleterious effects of oxidative stress in ageing, especially dysfunction of cardiac mitochondria, as well mitochondrial involvement in neurodegeneration. An attractive chapter concerns the implication of apoptosis, the programmed cell death, linked to cytochrome oxidase defect. Iatrogenic mitochondrial diseases are studied in Part II either due to a sen- sitivity to some drug or especially induced by antiretroviral drugs. Several drugs lead to a mitochondrial toxicity: antiepileptic treatment, anticancer drugs. Mitochondrial damage involving mainly the mitochondrial DNA is frequent in patients treated for several years by antiretroviral drugs, with absolute deple- tion of mitochondrial DNA. Some patients with severe lactic acidosis and mul- tiple organ failures may be improved by carnitine. Special problemes arise in newborn and in perinatal exposure to antiretroviral drugs. In case of treated mother a mitochondrial disease could occur in non infested newborn.