What do the results of the study mean for people with muscular dystrophy?
• Background Information- What are mitochondria and how are they involved in muscular dystrophy? • Further information and links: • References What did the new research in the USA show? Mitochondria are the batteries of cells, providing them with energy. Damage to the mitochondria can trigger the death of the whole cell. This mitochondrial damage involves the protein cyclophilin D and blocking the action of this protein can prevent the mitochondrial damage and subsequent cell death (you can read more background about this process at the end of this article). Professor Jeffery Molkentin’s group of researchers used the mouse models of limb girdle muscular dystrophy 2F, congenital muscular dystrophy 1A and Duchenne muscular dystrophy. In all three models it was shown that the mitochondria was swollen and damaged. Two different strategies were used to block cyclophilin D. Initially, the three mouse model strains were bred so that they also lacked the gene coding for cyclophilin D. In other
