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Heart saves muscle
May 26, 2009A heart muscle protein can replace its missing skeletal muscle counterpart to give mice with myopathy a long and active life, show Nowak et al. The findings will be published online on Monday, May 25, 2009 (www.jcb.org) and will appear in the June 1, 2009 print issue of the Journal of Cell Biology.
The contraction machinery protein, actin, exists in different forms in the adult heart and skeletal muscles. The heart form, ACTC, is also the dominant form in skeletal muscle of the fetus. But during development, the skeletal form, ACTA1, increases in production and by birth has taken over. It is not clear why the switch occurs, or why it doesn't occur in the heart, but it happens in every higher vertebrate and, for that reason, has been considered vitally important.
Mutations to the ACTA1 gene cause a rare but serious myopathy. Most patients die within the first year of life and some are born almost completely paralyzed. Mice lacking ACTA1 die nine days after birth. Nowak et al. wondered if ACTC could compensate for a lack of ACTA1. The two proteins differ only slightly but, like the developmental switch in production, this difference is conserved across species. Many researchers therefore assumed such compensation would never work.
But it did. Nowak and colleagues crossed Acta1 mutant mice with transgenic mice that express human ACTC at high levels in skeletal muscle cells. The resulting mice didn't die at nine days. In fact, almost all of them (93.5%) survived more than three months, and some more than two years. The mice's locomotor performance was comparable with wild-type, as was their overall muscle strength (though individual muscle fibers were slightly weaker), and their endurance was actually higher-they ran faster and for longer.
This begs the question, Why do we even have ACTA1? Besides pondering that, Nowak and colleagues are also working out how to boost endogenous ACTC as a possible therapy for ACTA1-lacking patients.
Rockefeller University Press
But it did. Nowak and colleagues crossed Acta1 mutant mice with transgenic mice that express human ACTC at high levels in skeletal muscle cells. The resulting mice didn't die at nine days. In fact, almost all of them (93.5%) survived more than three months, and some more than two years. The mice's locomotor performance was comparable with wild-type, as was their overall muscle strength (though individual muscle fibers were slightly weaker), and their endurance was actually higher-they ran faster and for longer.
This begs the question, Why do we even have ACTA1? Besides pondering that, Nowak and colleagues are also working out how to boost endogenous ACTC as a possible therapy for ACTA1-lacking patients.
Rockefeller University Press
Myopathy Current Events and Myopathy News RSSESC to give talks on Diabetes in three cities in China
As a result of successful events organised last year, a second Joint Scientific Forum, organised by the European Society of Cardiology (ESC) and the European Association for the Study of Diabetes (EASD), two of the most respected professional medical organisations in Europe, will be held from 27-29 November at three venues across China - Beijing, Shanghai and Guangzhou.
Muscle weakness a common side effect of long stays in intensive care units
After decades of focusing on the management of respiratory failure, circulatory shock and severe infections that lead to extended stays in hospital intensive care units, critical care researchers are increasingly turning attention to what they believe is a treatable complication developed by many who spend days or weeks confined to an ICU bed: debilitating muscle weakness that can linger long after hospital discharge.
Australian team reveals world-first discovery in a 'floppy baby' syndrome
In a world first, West Australian scientists have cured mice of a devastating muscle disease that causes a Floppy Baby Syndrome - a breakthrough that could ultimately help thousands of families across the globe.
Stem cell breakthrough gives new hope to sufferers of muscle-wasting diseases
An experimental procedure that dramatically strengthens stem cells' ability to regenerate damaged tissue could offer new hope to sufferers of muscle-wasting diseases such as myopathy and muscular dystrophy, according to researchers from the University of New South Wales (UNSW).
Potential therapy for congenital muscular dystrophy
Current research suggests laminin, a protein that helps cells stick together, may lead to enhanced muscle repair in muscular dystrophy.
Penn researchers discover 'modus operandi' of heart muscle protein
Researchers at the University of Pennsylvania School of Medicine have discovered that a protein called leiomodin (Lmod) promotes the assembly of an important heart muscle protein called actin. What's more, Lmod directs the assembly of actin to form the pumping unit of the heart. The findings appear in this week's issue of Science.
Massive microRNA scan uncovers leads to treating muscle degeneration
Researchers have discovered the first microRNAs - tiny bits of code that regulate gene activity - linked to each of 10 major degenerative muscular disorders, opening doors to new treatments and a better biological understanding of these debilitating, poorly understood, often untreatable diseases.
Researchers study the possible relationship between myopathies and coeliac disease
Inflammatory myopathies are immunological diseases that lead to inflammations in muscular tissue. As of yet, little is known about the cause of these myopathies, but it is believed to be an abnormal immune response by our bodies.
U of MN researchers develop mouse model for muscle disease
Researchers from the University of Minnesota have identified the importance of a gene critical to normal muscle function, resulting in a new mouse model for a poorly understood muscle disease in humans.
ESC Congress 2004: Trial supports early, aggressive statin use after heart attack
Patients suffering from acute coronary syndromes should be treated with cholesterol-lowering drugs known as statins early and aggressively, according to the results of an international clinical trial led by a team of investigators at the Duke Clinical Research Institute, the University of Texas Southwestern Medical Center and the Brigham and Women's Hospital and presented this afternoon at the ESC Congress 2004, Munich, Germany.
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