Sunday, 21 August 2011

ALS Cause and Treatment - A Possible Breakthrough

ALS (amyotrophic lateral sclerosis) is a devastating neurodegenerative disease which is nearly always fatal and until recently has been very difficult for scientists to understand. Without knowing the root cause of a disease it’s hard to find an effective treatment. Now researchers at Northwestern University in the United States report that they have found the problem that underlies ALS.

Amyotrophic lateral sclerosis is also known as Lou Gehrig’s disease after the famous baseball player who suffered from the illness. It's a horrible disease in which the motor neurons (nerve cells that control speaking, swallowing, breathing and movement) degenerate and die, while the thinking and reasoning areas in the brain are usually unaffected. ALS is one of a group of illnesses known as motor neuron diseases. Amyotrophic lateral sclerosis patients generally die within five years of the disease’s onset, although some patients are able to live longer. 

Three types of ALS have been discovered: familial ALS, which has a hereditary component, sporadic ALS, which is not inherited and is the most common ALS type, and a rarer kind of ALS that is accompanied by dementia. In this third type of amyotrophic lateral sclerosis both motor neurons and cells in the frontal lobe of the brain die. The frontal lobe is responsible for our higher mental functions.

The scientists at Northwestern University say that in all types of ALS proteins aren’t being recycled properly in the neurons of the brain and spinal cord. The problem seems to arise when a specific protein called ubiquilin2, which is normally responsible for the recycling of damaged proteins in neurons, stops doing its job. The ubiquilin2 and the damaged proteins collect in the neurons, causing them to degenerate.

The researchers say that the next step in their research is to find drugs that help improve protein recycling. Their discoveries may possibly help other disorders known to be due to the collection of abnormal proteins, such as Alzheimer’s disease and Parkinson’s disease.