Inflammation Clue To Fragile Bones In Muscular Dystrophy
Duchenne's muscular dystrophy is the most common of a group of genetic diseases when muscles become progressively weakened and wasted. It is caused by an abnormality of the dystrophin gene that is involved in the function of muscle cells. One in around 3,500 boys is affected. Little boys under the age of three will find walking, running and jumping difficult, and by the age of 11 they are unable to walk.
"These children have very fragile bones that fracture easily," said Dr Rufo at the European Symposium on Calcified Tissues in Vienna today (25 May). "We thought that this was due to mechanical failure - the load on the bones - but felt sure that other factors were involved," she explained.
The abnormal dystrophin gene causes muscle fibres to degenerate and become inflamed. As well as muscle damage, patients have osteoporosis and therefore a greater risk of fractures.
Dr Rufo's team at the University of L'Aquila found that children with DMD have increased levels of IL-6, a molecule that is also know to reduce bone formation and increase bone removal. In laboratory studies, the bone mass was reduced in dystrophin-deficient mice shown by the reduced bone forming cells (osteoblasts) and increased bone removal cells (osteoclast). When healthy osteoblasts were treated with sera collected from DMD patients they failed to mineralise the bone matrix and their IL-6 levels were increased. More than 100 other genes linked to osteoblast-osteoclast 'cross-talk' were also affected.
Their work is significant because glucocorticoids may be given to patients to help improve muscle strength and function for a short period of time. "Glucocorticoids are good for muscles in DMD but not so good for bones," said Dr Rufo. "Now we have found that osteoporosis in DMD patients could partly be explained by IL-6, we can try and find new ways to tackle inflammation," she concluded.
Source: Snell Communications Ltd
There are no references listed for this article.
Please use one of the following formats to cite this article in your essay, paper or report:
Ltd, Snell Communications. "Inflammation Clue To Fragile Bones In Muscular Dystrophy." Medical News Today. MediLexicon, Intl., 26 May. 2009. Web.
10 Dec. 2016. <http://www.medicalnewstoday.com/releases/151331.php>
Ltd, S. (2009, May 26). "Inflammation Clue To Fragile Bones In Muscular Dystrophy." Medical News Today. Retrieved from
Please note: If no author information is provided, the source is cited instead.
Contact our news editors
For any corrections of factual information, or to contact our editorial team, please see our contact page.
Copyright Medical News Today: Excluding email/sharing services explicitly offered on this website, material published on Medical News Today may not be reproduced, or distributed without the prior written permission of Medilexicon International Ltd. Please contact us for further details.