While many know this disease as Lou Gehrig’s disease, Amyotrophic Lateral Sclerosis has afflicted many thousands of individuals both before and after Gehrig developed the disorder. It is believed that perhaps 30,000 Americans currently suffer from this form of motor neuron disorder, which is often known by its initials, ALS.
ALS, as stated before, is a disease that affects the motor neurons, and these are neurons that exist in our central nervous system. There are several other neurological diseases that only affect these particular motor neurons, but ALS is by far the most common of these disorders. Typically, symptoms of the disease begin showing up between the ages of 40 to 60, however it can certainly occur in much younger individuals. There is no cure for ALS, and the disease is fatal. Most patients die within two or three years, and less than five percent will live beyond 10 years.
Often the initial symptom of ALS is muscle weakness, and this is because when the neurons begin to degenerate our muscles atrophy. Often a person with early stage ALS will experience weakness as well as not being able to control typical involuntary movements, such as lifting a cup of coffee or throwing a ball. Muscle twitching is another early symptom, as well as slurred speech, difficulty walking or running and problems with normal tasks such as writing, brushing teeth or buttoning a shirt.
Eventually, as the disease progresses, sufferers often have trouble moving at all and are confined to a wheelchair. Difficulty in speaking can become progressively worse, and difficulty swallowing also occurs as the disease progresses. Respiratory problems also occur in later stages, and those with ALS often are put on long-term automatic ventilators to help them breath. While there are some drugs available to help ease the symptoms, only one drug helps to slow progression of the disease, but typically survival rates are only increased by a matter of months to a few years.
In the 1990s, scientists found that is about 20% of ALS cases, a certain type of gene mutation was present, although they are not sure what causes the mutation. Inheriting ALS is rare but does happen occasionally, and this has been linked to a specific chromosome in the body. Within the last few years, a bigger discovery has targeted the protein identified as C9orf72 as the most likely culprit for ALS. The problem exists in one section of the protein. Typically this section will repeat about 30 times for most people, but with ALS patients, the repetition occurs many hundreds of times. No one yet knows why this repetition happens, but it does seem to cause the development of ALS.
So many diseases are caused by problems that exist within our cells at the protein level, which is why scientists are constantly studying different proteins. This helps them learn more about how diseases such as ALS, Alzheimer’s, Parkinson’s and other degenerative diseases develop. Studying protein involves the outsourcing of services such as peptide synthesis, N-terminal sequencing and protein synthesis. Biotech firms around the globe provide these helpful research services, and hopefully one day, cures and successful treatments will be developed.
Armand Zeiders enjoys blogging about biomedical research. For more information about protein sequencing service, or to find more biotechnology services, click here.
