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A New Target For Machado Joseph Disease Treatment

Machado-Josephdisease (MJD) is a hereditary neurodegenerative disorder that destroys the brainareas...

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Catarina AmorimRSS Feed of this column.

After many years as a scientist (immunology) at Oxford University I moved into scientific journalism and public understanding of science. I am still at Oxford Uni but now I write about any bio... Read More »

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Despite much research, the genetic causes why animals have such different longevities remain largely unknown, much because so many factors act on ageing that isolating the effect of a single gene is  almost  impossible.

But now, a study just published in the journal AGE might help to change that as researchers Pedro Magalhães and Yang Li from the Institute of Integrative Biology, at the UK University of Liverpool,  unveil a new method that has already help them to identify several  proteins involved in DNA-repair and in the recycling of abnormal molecules as being linked to longevity. 

The most abundant and important molecules in all living organisms are proteins; after all they manage to participate in every single one of life’s essential reactions. So it is easy to see why scientists have been making such a fuss trying to learn how to synthesise them in laboratory as this would provide them with a tool of extraordinary potential. Unfortunately, this has not proved easy.

Researchers from the University of Minho in Portugal have discovered that rats exposed before birth to glucocorticoids (GC) not only show several brain abnormalities similar to those found in addicts, but become themselves susceptible to addiction (the glucorticoids, which are stress hormones, were used to mimic pre-natal stress).  But even more remarkable, Ana João Rodrigues, Nuno Sousa and colleagues were able to reverse all the abnormalities  (including the addictive behavior) by giving the animals dopamine (a neurotransmitter/ brain chemical). 

A study out tomorrow in Nature by researchers from the Institute of Healthy Ageing at the University College of London and colleagues is questioning the anti-aging effects of sirtuin – which is “just”the most important anti-aging gene of the decade - claiming that its capacity to increase longevity was nothing more than an experimental error, and showing that,once the flaws are corrected, sirtuin has no effect on lifespan.

Cardiovascular diseases (CVD) already account for one third of all global deaths and raising, with effective heart regeneration therapies yet to be developed despite worldwide research efforts. But a new study by scientists from Oxford University and the University of Coimbra in Portugal might have put us a step closer with the discovery of the key molecule regulating the development of several heart and blood vessels’ tissues in the zebrafish embryo. 
Scientists have identified a biochemical abnormality behind the potentially fatal neurodegenerative Machado-Joseph disease (MJD) and, using several models of the disease, were able to reverse the problem in what may be a crucial step towards a cure for humans.  Currently, the disease is incurable and the patients’ increasing neurodegeneration cannot be stopped.