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Muscle disease symptom reversed | Muscle disease symptom reversed |
(about 23 hours later) | |
A key symptom of muscular dystrophy has been reversed by scientists in the lab. | A key symptom of muscular dystrophy has been reversed by scientists in the lab. |
They identified a compound that eliminated the prolonged muscle tensing known as myotonia in mice bred with a common form of the condition. | They identified a compound that eliminated the prolonged muscle tensing known as myotonia in mice bred with a common form of the condition. |
The researchers say the discovery raises hope myotonia could be reversed in muscular dystrophy patients even after it has become well established. | The researchers say the discovery raises hope myotonia could be reversed in muscular dystrophy patients even after it has become well established. |
The University of Rochester study is published online by the Journal of Clinical Investigation. | The University of Rochester study is published online by the Journal of Clinical Investigation. |
As we move forward, we should not be content to keep this condition from getting worse -we should set our set our sights on making it better. Dr Charles ThorntonUniversity of Rochester | As we move forward, we should not be content to keep this condition from getting worse -we should set our set our sights on making it better. Dr Charles ThorntonUniversity of Rochester |
Myotonic dystrophy - the most common form of muscular dystrophy in adults - is characterised by progressive muscle wasting and weakness, particularly in the lower legs, hands, neck, and face. | |
It undermines a key mechanism which controls electrical activity in the muscles, destroying their ability to relax and resulting in myotonia - often one of the first signs of disease. | It undermines a key mechanism which controls electrical activity in the muscles, destroying their ability to relax and resulting in myotonia - often one of the first signs of disease. |
This can be particularly severe in the hand muscles, causing a patient's grip to lock. | This can be particularly severe in the hand muscles, causing a patient's grip to lock. |
Relaxation restored | Relaxation restored |
The Rochester team designed a molecule that, when injected into mice with myotonic dystrophy, restored a critical control mechanism, and with it the ability of muscles to relax. | The Rochester team designed a molecule that, when injected into mice with myotonic dystrophy, restored a critical control mechanism, and with it the ability of muscles to relax. |
Researcher Dr Charles Thornton, director of the university's Neuromuscular Disease Center, said: "This work should provide hope and encouragement to people with myotonic dystrophy and their families. | Researcher Dr Charles Thornton, director of the university's Neuromuscular Disease Center, said: "This work should provide hope and encouragement to people with myotonic dystrophy and their families. |
"It encourages us to believe that other parts of the disease could be reversible as well. | "It encourages us to believe that other parts of the disease could be reversible as well. |
"As we move forward, we should not be content to keep this condition from getting worse. We should set our set our sights on making it better." | "As we move forward, we should not be content to keep this condition from getting worse. We should set our set our sights on making it better." |
The Rochester team pinpointed a genetic flaw responsible for myotonia five years ago. | The Rochester team pinpointed a genetic flaw responsible for myotonia five years ago. |
This disrupts the intricate and finely balanced flow of tiny charged particles responsible for electrical activity in cells. | This disrupts the intricate and finely balanced flow of tiny charged particles responsible for electrical activity in cells. |
One key "channel" is effectively disabled, causing electrical signals in the muscles to stay jammed in the "on" mode. | One key "channel" is effectively disabled, causing electrical signals in the muscles to stay jammed in the "on" mode. |
The compound - morpholino - developed by the researchers in collaboration the biotechnology company Gene Tools, restores the proper genetic instructions for building the disabled channel. | The compound - morpholino - developed by the researchers in collaboration the biotechnology company Gene Tools, restores the proper genetic instructions for building the disabled channel. |
Prolonged effect | Prolonged effect |
When it was injected into mice muscle cells the channel was restored, and the myotonia all but disappeared. | When it was injected into mice muscle cells the channel was restored, and the myotonia all but disappeared. |
Dr Thurman Wheeler, who led the research, said: "This material is incredibly stable in the cells once we get it inside the muscle. | Dr Thurman Wheeler, who led the research, said: "This material is incredibly stable in the cells once we get it inside the muscle. |
"And the effects are surprisingly prolonged, which makes it potentially more attractive as a treatment." | "And the effects are surprisingly prolonged, which makes it potentially more attractive as a treatment." |
However, the Rochester team said more work was needed before this new approach could be tested in people. | However, the Rochester team said more work was needed before this new approach could be tested in people. |
The first priority will be to develop a better method of delivering the compound throughout the body. | The first priority will be to develop a better method of delivering the compound throughout the body. |
Dr Marita Pohlschmidt, director of research at the Muscular Dystrophy Campaign, described the research as "exciting and reassuring". | Dr Marita Pohlschmidt, director of research at the Muscular Dystrophy Campaign, described the research as "exciting and reassuring". |
But she added: "We must be reminded that these experiments have been carried out in an animal model. | But she added: "We must be reminded that these experiments have been carried out in an animal model. |
"We do however look forward to further research to see whether this kind of approach can be found to have an effect in people with the condition." | "We do however look forward to further research to see whether this kind of approach can be found to have an effect in people with the condition." |
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