Investigating the Living World (SCIE - 6662S - 2)----- Week 3

Ask a Scientist

About four years ago my niece died from Amyotrophic lateral sclerosis, ALS or Lou Gehrig’s disease, at the age of nineteen. She was diagnosed at age seventeen; the disease progressed very quickly and took her from us at a young age. ALS is not as common in young people; it tends to strike people between 40 and 60 years of age with men being affected more often than women.  In 90 to 95 percent of all ALS cases the disease occurs with no clearly associated risk factors. People that have been diagnosed with ALS typically do not have a family history of the disease, and their family members are not considered to be at increased risk for developing ALS. (National Institute of Neurological Disorders and Stroke, 2012). In the wake of my nieces’ death I did some of my own research on the disease to try and understand it better. Motor neurons have been the focus of ALS research; this is based on the symptoms of this disease involving weakness of muscles (ALS Association, 2010). The research for treatment of ALS deals with the fate of motor neurons. The thought is that it might be sufficient to implant, by gene or stem cell therapy, a minimum number of cells that do not have any mutant protein but instead make helpful factors. The mutant protein, copper-zinc superoxide dismutase (SOD1), is linked to some cases of the disease. If mutant SOD1 is present in motor neurons, but normal SOD1 is in the surrounding, protective glia cells, then the motor neurons do not die. (ALS Association, 2010).

My question is this: If treatment research focuses on the interaction between motor neurons and non-neuronal cells and how much motor neuron death depends on neighboring cells, then how will non-neuronal cells protect motor neurons expressing an ALS-causing mutation.

I have not yet received my response so there will be more to come at a later date.

References

ALS Association. (2010). Cell Targets. Retrieved from http://www.alsa.org/research/about-als-research/cell-targets.html

National Institute of Neurological Disorders and Stroke. (2012, December 20). Amyotrophic Lateral Sclerosis (ALS) Fact Sheet. Retrieved from http://www.ninds.nih.gov/disorders/amyotrophiclateralsclerosis/detail_ALS.htm#222914842