Cause of Lou Gehrig's Disease Identified

Researchers investigating Lou Gehrig's disease may very well be considering themselves "the luckiest [people] on the face of this earth." According to a recent study, researchers believe they have found the cause of the disease,

The study, published in the peer-reviewed scientific journal Cell Stem Cell, details how researchers indentified what they believe is the cellular cause of Amyotrophic lateral sclerosis (ALS), also known as Lou Gehrig's disease.

The researchers from the University of Wisconsin-Madison achieved this after extensively studying proteins that erect transport structures -- called neurofilaments -- inside motor neurons. These neurofilaments move chemicals and cellular parts to the far sides or nerve cells, allowing for the neurological communication that results in the moving of our limbs.

Prior to this most recent study, research already knew that misallocated proteins in a nerve could block communication along nerve fibers, eventually resulting in the nerve fiber malfunctioning and dying.

The symptoms of ALS result from this neurological disaster, where loss of motor control, paralysis, and eventually death occur.

However, this most recent research looks deeper into the cause of ALS, determining just why this protein misallocation occurs.

Using induced pluripotent stem (iPS) cells, the researcher were able to make numerous lab-grown motor neurons to expensively study the development of ALS.

Long-term observation eventually resulting in the research team discovering that a shortage of one of three proteins in the neurofilament results in the proteins erecting poorly formed structures and thus cannot be transported easily. Structures lost in transport eventually lead to "tangles" along neuronal pathways that inhibit neurological communication and lead to ALS.

Dr. Su-Chun Zhang, senior author and neuroscientist at UW-Madison, is encouraged by this discovery, explaining that it takes experts one step closer to developing an appropriate treatment for Lou Gehrig's disease, which affects an estimated 30,000 people in the U.S. alone.

"Nobody knew this before, but we think if you can target this early step in pathology, you can potentially rescue the nerve cell," he explained in auniversity press release.

The study was published in Cell Stem Cell, a Cell publication, on April 3.