Improved clearance of unwanted proteins may be beneficial in MND
03 December 2007
One of the classical hallmarks of motor neurone disease as seen down the microscope is the presence of clumps – so called aggregates – of proteins.
How these proteins form and how they are removed in MND was one of the topics of a scientific session of on the last day of the International Symposium on ALS/MND.
It opened by Prof David Rubinstein giving a clear and elegant overview of the role of a process known as autophagy in the clearing of these clumps of proteins. “We want to enhance the clearance of the poison within motor neurones” he explained. Autophagy is a process of engulfing the damaging proteins, once engulfed they are then digested and spat out of the cell.
In a mouse model of Huntingdon’s disease researchers have shown that drugs that increase the levels of autophagy in the cell are beneficial to the health and survival of the animals. Thus, if this approach can work in Huntingdon’s disease then it may work for MND too.
Further evidence of a compromise in the ability to clear unwanted proteins in MND was presented by Dr Caterina Bendotti from Milan. She showed that parts of an alternative system of rubbish collection were altered in a mouse model of ALS/MND.
More research in these areas will help researchers to develop targets of drugs to improve the clearance of rubbish from motor neurones in MND.
How these proteins form and how they are removed in MND was one of the topics of a scientific session of on the last day of the International Symposium on ALS/MND.
It opened by Prof David Rubinstein giving a clear and elegant overview of the role of a process known as autophagy in the clearing of these clumps of proteins. “We want to enhance the clearance of the poison within motor neurones” he explained. Autophagy is a process of engulfing the damaging proteins, once engulfed they are then digested and spat out of the cell.
In a mouse model of Huntingdon’s disease researchers have shown that drugs that increase the levels of autophagy in the cell are beneficial to the health and survival of the animals. Thus, if this approach can work in Huntingdon’s disease then it may work for MND too.
Further evidence of a compromise in the ability to clear unwanted proteins in MND was presented by Dr Caterina Bendotti from Milan. She showed that parts of an alternative system of rubbish collection were altered in a mouse model of ALS/MND.
More research in these areas will help researchers to develop targets of drugs to improve the clearance of rubbish from motor neurones in MND.
