Navigation Links
Clues to the Mystery of Lou Gehrig's Disease

Researchers have recently reported clues to unraveling the mystery of the disease best known for striking down New York Yankees first baseman Lou Gehrig nearly 70 years ago//.

According to Virginia Lee, Ph.D., of the University of Pennsylvania a team of researchers has identified the misfolded protein that causes both Lou Gehrig's disease or amyotrophic lateral sclerosis (ALS), as well as a less well-known disease, frontotemporal lobar degeneration.

The findings of the study, published in the Oct. 6 issue of Science identify it as TAR DNA-binding protein 43 (TDP-43), that has several functions and is found in the nucleus of many cell types.

Misfolded proteins are a common occurrence in neurodegenerative diseases. They are tagged for recycling by the protein ubiquitin, but instead of being broken down they are dumped in the neurons.

However Dr Lee said that the specific ubiquitinated protein involved in ALS and frontotemporal lobar degeneration had not been earlier identified. She and her colleagues noted that the finding "resolves a long-standing enigma."

Clinical features such as dementia and motor complications are common to both ALS and frontotemporal lobar degeneration patients which made researches consider the likelihood of the two diseases being related in some way.

Tests using tissue samples showed that the motor neurons of ALS patients and the frontal and temporal neurons of frontotemporal lobar degeneration patients contain large amounts of ubiquitinated TDP-43 in their cytoplasm.

The researchers therefore concluded that this finding implied that ALS, frontotemporal lobar degeneration, and possibly other neurodegenerative diseases are part of "a spectrum of disorders that share similar pathological mechanisms, culminating in the progressive degeneration of different selectively vulnerable neurons."

Dr Lee said, "It's very exciting that we finally made the connection between dem entia and motor neuron disease. Clinically there's overlap in these two disorders, so it was very tantalizing to see if there was anything to link them biochemically."

The researchers developed monoclonal antibodies to TDP-43, and tested them in 72 post-mortem tissue samples patients with either ALS or frontotemporal lobar degeneration and found that all 72 cases contained misfolded TDP-43. Dr Lee said, "Since many cases were studied, the data became very compelling."

According to study co-author John Trojanowksi, M.D., Ph.D., director of Penn's Institute on Aging although ALS is well-known because of its link with Gehrig, frontotemporal lobar degeneration is less well-known while this may be because of its rarity in spite of it being the second most common cause -- after Alzheimer's disease -- of dementia in people under the age of 65.

Dr. Trojanowksi said, "Another reason for (its) relative obscurity is also undoubtedly the shame family members experience with the strange and, at times, bizarre behavior of patients, including disturbing obsessions, larceny, or even sexual deviancy, that may bring the patient to the attention of the legal system rather than to the healthcare system."

Dr Lee pointed out that usefulness of the finding depends on whether future research shows that TDP-43 is a useful target for new therapies and diagnostic tools.
NLA
'"/>




Related medicine news :

1. Innovative yeast genome may handle Cancer Clues
2. Researchers Discover New Clues To Identify Tooth Decay
3. Leeches Provide Clues Regarding The Prevention Of Heart Disease
4. Excavations Reveal Clues Of Early Warfare
5. Sleeping Computers Yield Clues About Genetic Disorders
6. Clues To Identify Psychological Seizures
7. A Human Brain Gene Could Hold Clues On The Evolution Of Brain Capacity
8. Clues Found On How Deadly Bacterium Gains Foothold
9. Clues to Allay Aging
10. Regrowth of Zebrafishs Tail Fin may Offer Clues for Treating Human Injuries
11. Renegade RNA: Clues to Cancer and Normal Growth
Post Your Comments:
*Name:
*Comment:
*Email:


(Date:10/13/2017)... ... October 13, 2017 , ... ... Software Development, has been awarded a contract by the Center for Medicare and ... aims to accelerate the enterprise use of Agile methodologies in a consistent and ...
(Date:10/12/2017)... Del. (PRWEB) , ... October 12, 2017 , ... ... and advisory services for healthcare compliance program management, will showcase a range of ... National Association for Assisted Living (NCAL) Convention and Expo to be held October ...
(Date:10/12/2017)... ... October 12, 2017 , ... The company has developed a ... and regulatory authorities worldwide. From Children’s to Adults 50+, every formula has been ... standard. , These products are also: Gluten Free, Non-GMO, Vegan, Soy Free, ...
(Date:10/12/2017)... ... October 12, 2017 , ... Asante, ... post-acute health care, have expanded their existing home health joint venture through an ... has been operating a joint venture home health company with Asante, delivering clinically ...
(Date:10/12/2017)... (PRWEB) , ... October 12, 2017 , ... HMP ... the recipient of a 2017 Folio Magazine Eddie Digital Award for ‘Best B-to-B Healthcare ... City on October 11, 2017. , The annual award competition recognizes editorial and design ...
Breaking Medicine News(10 mins):
(Date:9/28/2017)... , Sept. 28, 2017 Cohen Veterans Bioscience ... the use of wearable and home sensors for real-time ... Early Signal Foundation, a nonprofit organization focused on disruptive ... provide an affordable analytical system to record and integrate ... ...
(Date:9/25/2017)... R.I. , Sept. 25, 2017  EpiVax, ... assessment, vaccine design, and immune-engineering today announced the ... on the development of personalized therapeutic cancer vaccines. ... has provided exclusive access to enabling technologies to ... Eng., MBA will lead EpiVax Oncology as Chief ...
(Date:9/19/2017)... , Sept. 19, 2017 HistoSonics, Inc., a venture-backed medical device company developing ... of targeted tissues, announced three leadership team developments today:   ... ... ... Veteran medical device executive Josh Stopek ...
Breaking Medicine Technology: