(HOME) 

Subject: MD/chelator/iron

    ______________

  

   Med Hypotheses 13: 153-60 (1984)[84190712]

Proposed treatment of Duchenne muscular dystrophy with desferrioxamine.



    I. A. Clark



   The primary disturbance in Duchenne muscular dystrophy (DMD) appears
   to affect membrane function, and changes characteristic of
   oxidant-induced damage occur in skeletal muscle and erythrocytes.
   There is recent evidence that DMD is a functional tocopherol
   deficiency, with reduced levels of the lipoprotein required to carry
   tocopherol to tissues. This may explain the parallels between DMD and
   dietary tocopherol deficiency. Thus DMD should follow the usual
   experience of other examples of oxidative pathology, where the balance
   between tocopherol, the main antioxidant in membrane lipids, and non
   protein-bound iron, an important catalyst of reactions which produce
   oxidizing free radicals, largely determines whether or not tissue
   damage occurs. Desferrioxamine prevents oxidant damage in vitro and in
   vivo by removing this iron, and may therefore be able to reverse the
   muscle damage of DMD. Recent experience with this drug in long term
   dialysis patients is consistent with this suggestion.

   MeSH Terms:
     * Animal
     * Deferoxamine/therapeutic use
     * Genes, Recessive
     * Human
     * Iron/metabolism
     * Linkage (Genetics)
     * Membrane Lipids/metabolism
     * Muscles/metabolism
     * Muscular Dystrophy/drug therapy
     * Muscular Dystrophy/etiology
     * Muscular Dystrophy/genetics
     * Muscular Dystrophy, Animal/metabolism
     * Oxidation-Reduction
     * Support, Non-U.S. Gov't
     * Vitamin E/metabolism
     * Vitamin E Deficiency/complications



   Substances:
     * Iron
     * Vitamin E
     * Membrane Lipids
     * Deferoxamine


     _________________________________________________________________
 (HOME)