Increased Oxidation of Tocopherols in Chickens with Inherited Muscular Dystrophy

Abstract

Inherited muscular dystrophy in chickens shares many characteristics with inherited muscular dystrophy in humans. Both are conditions caused by the defect of a single recessive gene, both lead to the degeneration of muscle tissue over time, and the underlying biochemical basis for cell death is unknown in both disorders. Numerous hypotheses have seen suggested to explain muscular dystrophy.¹ One of these postulates the basic biochemical defect of inherited muscular dystrophy is one which causes oxidative stress in muscle tissue, and that this is the underlying cause of cell death.² Table 1 summarizes the changes found in dystrophic muscle from both chickens and humans that closely resemble those caused in muscle by oxidative processes.¹ Oxidative stress in muscle cells could be caused by two basic biochemical defects. First, the defect could be one which leads to the excessive production of free radicals in muscle tissue. Second, the defect could be in one of the antioxidant systems important to normal muscle integrity, although apparently not in any of the antioxidant systems analyzed to date, all of which show enhanced activity in dystrophic muscle.¹