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
