Navigation Links
Study identifies key player in motor neuron death in Lou Gehrig's disease
Date:3/26/2014

Amyotrophic lateral sclerosis, also known as Lou Gehrig's disease, is marked by a cascade of cellular and inflammatory events that weakens and kills vital motor neurons in the brain and spinal cord. The process is complex, involving cells that ordinarily protect the neurons from harm. Now, a new study by scientists in The Research Institute at Nationwide Children's Hospital points to a potential culprit in this good-cell-gone-bad scenario, a key step toward the ultimate goal of developing a treatment.

Motor neurons, or nerve cells, in the brain and spinal cord control the function of muscles throughout the body. In amyotrophic lateral sclerosis (ALS), motor neurons die and muscles weaken. Patients gradually lose the ability to move and as the disease progresses, are unable to breathe on their own. Most people with ALS die from respiratory failure within 3 to 5 years from the onset of symptoms.

For the study, published online this month in Neuron, researchers examined a protein involved in transcriptional regulation, called nuclear factor-kappa B (NF-κB), known to play a role in the neuroinflammatory response common in ALS. NF-ƘB has also been linked to cancer and a number of other inflammatory and autoimmune diseases.

Using animal models, the researchers studied disease progression in mice in which NF-ƘB had been inhibited in two different cell types astrocytes, the most abundant cell type in the human brain and supporters of neuronal function; and microglia, macrophages in the brain and spinal cord that act as the first and main form of defense against invading pathogens in the central nervous system. Inhibiting NF-ƘB in microglia in mice slowed disease progression by 47 percent, says Brian Kaspar, MD, a principal investigator in the Center for Gene Therapy at Nationwide Children's and senior author of the new study.

"The field has identified different cell types in addition to motor neurons involved in this disease, so one of our approaches was to find out what weapons these cells might be using to kill motor neurons," Dr. Kaspar says. "And our findings suggest that the microglia utilize an NF-κB-mediated inflammatory response as one of its weapons."

Inhibiting the protein in astrocytes had no impact on disease progression, so the search for the weapons that cell type uses against motor neurons continues. These preliminary findings also don't tell scientists how or why NF-κB turns the ordinarily protective microglia into neuron-killing molecules. But despite the mysteries that remain, the study moves scientists closer to finding a treatment for ALS.

The search for an ALS therapy has been focused in two directions: identifying the trigger that leads to disease onset and understanding the process that leads to disease progression. Changes in motor neurons are involved in disease onset, but disease progression seems to be dictated by changes to astrocytes, microglia and oligodendrocytes. Some cases of ALS are hereditary but the vast majority of patients have no family ties to the disease. The complexity of the disease and the lack of a clear familiar tie make screening before disease onset nearly impossible, highlighting the importance of slowing the disease, Dr. Kaspar says.

"Focusing on stopping the changes that occur in astrocytes and microglia has clinical relevance because most people don't know they're getting ALS, says Dr. Kaspar, who also is an associate professor of pediatrics and neurosciences at The Ohio State University College of Medicine. "We have identified a pathway in microglia that may be targeted to ultimately slow disease progression in ALS and are exploring potential therapeutic strategies and may have broader implications for diseases such as Alzheimer's and Parkinson's Disease amongst others."


'/>"/>

Contact: Gina Bericchia
Gina.Bericchia@NationwideChildrens.org
614-355-0487
Nationwide Children's Hospital
Source:Eurekalert

Related medicine news :

1. Study Suggests Vaccine May Help Kids With Brain Cancer
2. Study reveals how cancer drug causes diabetic-like state
3. Coffee Drinking in Pregnancy Wont Lead to Sleepless Baby: Study
4. Lower GI problems plague many with rheumatoid arthritis, Mayo Clinic study finds
5. Veggies Like Broccoli, Cabbage May Help Fight Breast Cancer: Study
6. No Added Cancer Risk From Hip Replacement Materials: Study
7. Reported Decline in U.S. Pneumonia Deaths May Be False: Study
8. Early Study Finds Some Promise for Lung Cancer Vaccine
9. Narcissists Often Ace Job Interviews, Study Finds
10. Sexual objectification of female artists in music videos exists regardless of race, MU study finds
11. Soy may alleviate hot flashes in menopause, large-scale study finds
Post Your Comments:
*Name:
*Comment:
*Email:
(Date:1/13/2017)... ... January 13, 2017 , ... The 18th European Congress: Perspectives in Lung ... 3-4, 2017. This Congress is expertly designed to meet the educational needs of ... lung cancer. , Chaired by Dr. Giorgio V. Scagliotti, Professor of Oncology at the ...
(Date:1/13/2017)... Princeton, NJ (PRWEB) , ... January 13, 2017 , ... ... of sale companions, will be showcasing the next generation companion scanner and data collector ... City. The new KDC270 has been created as an answer to the market’s need ...
(Date:1/13/2017)... PITTSBURGH, PA (PRWEB) , ... January 13, 2017 , ... An inventor from Pahrump, Nev., ... many others. "My urologist had me wear a Foley catheter and urine bag for a ... difficult and uncomfortable, so I decided that there should be a better way to do ...
(Date:1/13/2017)... ... January 13, 2017 , ... People who have sensitive ... of pain whenever they brush their teeth. Sadly, most dental hygiene products in the ... gums and teeth. For these people, continuing their daily oral care routine to keep ...
(Date:1/13/2017)... ... January 13, 2017 , ... Friday, January 13 at 5 pm, America’s Incredible ... the size of the location to 90,000 square feet. , Included in the expansion ... takes customers on an educational tour of the historic route, with each hole in ...
Breaking Medicine News(10 mins):
(Date:1/16/2017)... Tenn. , Jan. 16, 2017  Cumberland Pharmaceuticals ... of Kenneth J. Krogulski , CFA to its ... Executive Officer of Berkshire Asset Management LLC . ... a 30-year-old independent SEC registered investment advisory firm with ... has over 37 years, experience in security analysis and ...
(Date:1/15/2017)... Le conseil d,administration de SurgaColl Technologies Ltd., le ... régénération de tissus humains, annonce la nomination de William ... immédiat. ... Bill est un dirigeant expérimenté à l,international dans l,industrie des ... et de direction au sein de sociétés internationales spécialisées dans ...
(Date:1/13/2017)... 13, 2017 /PRNewswire/ - InMed Pharmaceuticals, Inc. ("InMed") (CSE: IN; OTCQB: ... Corporate Finance and Investment Banking at Eli Lilly & Company, ... Mr. Bott brings over 28 years of senior financial and ... ... has established a significant leadership position in financial and ...
Breaking Medicine Technology: