Lou Gehrig Disease (ALS) Cause and Origin Discovered

The potential origin and cause of Lou Gehrig’s disease (ALS)  may have been discovered by a group of scientists. The disease which currently has no cure is a disease whereby the part of the nervous system controlling voluntary movement is affected.

lou gehrigs

What really causes ALS?

In critical stages,  ALS may lead to paralysis, ultimately causing death of the patient. Broadly speaking, the motor neurones which send messages to the muscles involved in voluntary movements die out so that one can no longer voluntarily move the associated limbs because the information needed to allow for the movement of the muscles are never sent to the muscles concerned. The cause behind this degeneration of nerve cells has so far remained unknown.

A new study has however brought forward a possible explanation. Scientists have observed a phenomenon that could account for the malfunctioning of the nervous system. Having previously not deciphered the triggering mechanism of the disease, finding a cure to it has been challenging. Hopefully, this study could accelerate things in the right direction.

Faulty protein behind ALS?

A faulty protein formation could be the reason behind the disruption of the motor neurones. A genetic abnormality was previously found in a small group of patients suffering from Lou Gehrig’s disease whereby a gene mutation was observed. That gene was then transferred to animals to do testing for drugs – approach which did not work out. The authors of the recent study have therefore decided to study the cells containing the disease. They grew the cells in laboratory. They then concentrated on the proteins involved in the normal functioning of the motor neurones.

Along motor neurones travel electrical impulses. These occur in a network of nerve cells joined to each other. For the message to be relayed neurone to neurone, a substance called a neurotransmitter is needed. A neurotransmitter is essentially a chemical required to transfer information from parts of the neurones to others. Now, these neurotransmitters and other cellular parts involved in the network need a microfilament to be able to move about from their source to their destination. A microfilament acts as a transport vehicle, carrying the neurotransmitters to where they are needed. These microfilaments are made from protein molecules. Now, proteins are specific: they have a very particular shape that directs their activities. If this particular shape is distorted out of its normal one, the protein will not be able to carry out its function. Misshapen proteins constitute an enormous block along the nerve’s pathways because they cause tangles. The smooth circulation of messages from neurone to neurone is hence blocked, which leads to paralysis.

Chain of problems resulting from one mishap

What are the source of these tangles? The recent study found out that a protein is lacking in the neurofilament which results in the tangling-effect. When proteins are not of the appropriate shape, they cannot be easily transported to their site of action, which is at the roots of the problems. One protein missing leads to the formation of a distorted one, which in turn leads causes problems in the manufacture of microfilaments, which then impacts on the transport of neurotransmitters which thus affects the transmission of information from neurone to neurone. A chain of problems thus follow from just one trouble back in the making of proteins.

One of the authors of the study summed up the cause of the disease as being: “misregulation of one step in the production of the neurofilament.”

Hope added on hope

This malfunction occurs at the very onset of the disease. If this problem is dealt with at the earliest stage of the disease, the motor neurones can perhaps be saved from dying as a consequence. Now coming next is how to rescue the motor neurones? Question answered by the researchers. They managed to modify the gene that caused the malformation of the protein in question such that the nerve cells then appeared to be normal. Now, they are currently testing any potential drug that could do the trick.

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