Hoarding Junk is Hard on the Nerves in Neurodegenerative Diseases

Published: 2010-04-14, Last Modified:
Degenerative Brain Diseases Hoarding Junk

The wiring in our bodies that makes up our nervous system includes an immense number of nerve cells. In fact, some 1 billion neurons exist in our spinal cord alone and roughly 100 billion neurons exist in each of our brains. These nerve cells enable us to see, to breath, to move and to think. They also work together to control the beating of our hearts. With so many roles in the control of our bodies, failure of these cells to operate correctly leads to a wide variety of diseases.

One group of diseases that affects nerve cells is described as neurodegenerative. Often considered some of the most tragic diseases, neurodegenerative disorders are those where nerve operation degrades slowly over a number of years. The results for patients are typically profound as these diseases can rob a person of sight, mobility, the ability to breath or even the ability to think.

The identities of many diseases in this category are quite familiar to many of us with names such as Huntington’s Disease, Parkinson’s Disease, Lou Gehrig’s Disease and Alzheimer’s. Less familiar diseases under the same banner include Batten Disease and Kennedy’s Disease.

In their research efforts to try to understand these conditions, scientists have, in the last few years, found that the cause for nerve degradation and death in many of these diseases is faulty production and cleanup of materials within the nerve cells.

As an example, researchers at Ruhr University Bochum in Germany, found one cause of Parkinson’s Disease to be related to the accumulation of iron within brain nerve cells. This accumulation occurs when the disease affects the cleanup capabilities of these cells, limiting their ability to remove excess iron. Too much iron hanging around results in excess free radical damage to the nerve cells themselves.


Another example of a problem associated with cell cleanup is the failure of a cell to remove damaged material. The disease in this case is one with the unfamiliar name Infantile Neuronal Ceroid Lipofuscinosis (LINCL).

Researchers at Weill Cornell Medical College found that in children with this condition, the proteins and fats that normally become damaged by free radicals are not removed from the cells. The accumulation of this damaged material interferes with the ability of the neuron to do its job. While aging normally causes similar accumulation of such waste material, the genetic defect in this particular disease means more of this material collects faster because the cleanup ability of the cell is affected.

From various research sources, it appears many materials can accumulate in our nerve cells as a result of disease. In yet another related neurodegenerative disease, Niemann-Pick type C, brain cells die as a result of fat build up. However, protein accumulation in the brain cells seems to be the main cause of a great many neurodegenerative illnesses that involve poor cell waste management services.

In Kennedy’s Disease, deformed proteins cause the death of nerve cells responsible for muscle control. Alzheimer’s appears to be related to accumulation of fibrous proteins. Only recently, researchers at Albert Einstein College of Medicine found that Huntington’s Disease is likely the result of too many deformed proteins collecting in brain cells. Similarly, production of toxic proteins and a failure of the cell to remove these proteins is thought to be the cause of Lou Gehrig’s Disease or Amyotrophic Lateral Sclerosis (ALS).

So why do these nerve cells fail to clean out unwanted materials? For these conditions mentioned here, the problems generally appear genetic in nature. In some cases, mutated genes causing construction of poorly formed materials that are not recognized by the cell cleaning staff. In other cases, the genetic defects limit the effectiveness of the cleaners to remove materials that would normally accumulate. In either case, when materials do build up in the nerves, the result is less effective nerve cells or death of the nerve cells.

With this knowledge in mind, researchers are looking at ways to prevent the cells from creating toxic materials on one side and ensuring effective cell cleanup on the other. An important approach being researched in numerous institutions involves reprogramming the genes. A future article will cover the use of gene therapy in seeking treatment for many neurodegenerative diseases.

Related Links

http://faculty.washington.edu/chudler/facts.html
http://www.research-in-germany.de/31248/2009-08-21-parkinson-s-disease-iron-accumulation-to-the-point-of-demise,sourcePageId=12290.html
http://www.liebertpub.com/prdetails.aspx?pr_id=648
http://www.ucsfchildrenshospital.org/news/2004/07/neurodegenerative_disease_in_children_might_respond_to_neuro.html
http://www.uphs.upenn.edu/news/news_releases/2008/05/daph-dismantles-proteins.html
http://esciencenews.com/articles/2009/08/12/mighty.mice.treatment.targeted.muscle.improves.motor.neuron.disease
http://www.einstein.yu.edu/home/news.asp?id=480
http://www.eurekalert.org/pub_releases/2008-05/cshl-goa052808.php

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