Animal Models for Mitochondrial Disease

Although a variety of degenerative diseases are now known to be caused by two mutations in mitochondrial genes, the pathophysiology of these diseases remains poorly understood. As a consequence, relatively little progress has been made in developing new therapies for mitochondrial diseases. What has been needed are animal models for these diseases that are amenable to detailed biochemical, physiological, and molecular analysis, and on which promising therapies can be tested. In the past 5 yr, this deficiency has begun to be addressed by the construction of a number of mouse models of mitochondrial