TDP 43 News and Research

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Combination of sugar and polyphenols may prevent occurrence of neurodegenerative disease

Catherine Aaron and Gabrielle Beaudry were 17 when they knocked on the door of the laboratory of Alex Parker, a neuroscience researcher at the University of Montreal Hospital Research Centre. While students at Collège Jean-de-Brébeuf in Montreal, they were looking for a mentor for an after-school research project. Two and half years later, the results of this scientific adventure were published today in the Journal of Agricultural and Food Chemistry.

13 Apr 2016

Study suggests link between human endogenous retroviral genes and ALS

Scientists at the National Institutes of Health discovered that reactivation of ancient viral genes embedded in the human genome may cause the destruction of neurons in some forms of amyotrophic lateral sclerosis (ALS).

1 Oct 2015

Dementia sufferers may begin to lose awareness of memory problems 2-3 years before onset

People who will develop dementia may begin to lose awareness of their memory problems two to three years before the actual onset of the disease, according to a new study published in the August 26, 2015, online issue of Neurology, the medical journal of the American Academy of Neurology.

27 Aug 2015

Johns Hopkins researchers discover role of TDP-43 protein in autopsy brain cells of patients with ALS

Autopsies of nearly every patient with the lethal neurodegenerative disorder amyotrophic lateral sclerosis (ALS), and many with frontotemporal dementia (FTD), show pathologists telltale clumps of a protein called TDP-43. Now, working with mouse and human cells, Johns Hopkins researchers report they have discovered the normal role of TDP-43 in cells and why its abnormal accumulation may cause disease.

7 Aug 2015

New fly model developed to study motor neuron degeneration

Researchers at the Babraham Institute and University of Massachusetts Medical School in the United States have developed a new model to study motor neuron degeneration and have used this to identify three genes involved in the neurodegeneration process. These findings could have relevance for understanding the progression of amyotrophic lateral sclerosis (ALS) and other forms of motor neuron disease (MND).

7 Aug 2015

U of T researchers discover new details about key gene involved in ALS

A University of Toronto research team has discovered new details about a key gene involved in ALS, perhaps humanity's most puzzling, intractable disease.

16 Jul 2015

Immune system linked to death of motor neurons in Amyotrophic lateral sclerosis

A previously unknown link between the immune system and the death of motor neurons in Amyotrophic lateral sclerosis (ALS), also known as Lou Gehrig's disease, has been discovered by scientists at the CHUM Research Centre and the University of Montreal. The finding paves the way to a whole new approach for finding a drug that can cure or at least slow the progression of such neurodegenerative diseases as ALS, Alzheimer's, Parkinson's and Huntington's diseases.

10 Jun 2015

Mayo Clinic scientists create mouse model of ALS, FTD caused by mutations in C9ORF72 gene

Scientists at Mayo Clinic, Jacksonville, Florida created a novel mouse that exhibits the symptoms and neurodegeneration associated with the most common genetic forms of frontotemporal dementia (FTD) and amyotrophic lateral sclerosis (ALS, Lou Gehrig's disease), both of which are caused by a mutation in the a gene called C9ORF72.

24 May 2015

Hypermethylation serves as protective barrier inhibiting development of ALS, FTD

Penn Medicine researchers have discovered that hypermethylation - the epigenetic ability to turn down or turn off a bad gene implicated in 10 to 30 percent of patients with Amyotrophic Lateral Sclerosis (ALS) and Frontotemporal Degeneration (FTD) - serves as a protective barrier inhibiting the development of these diseases.

21 Mar 2015

TauRx achieves target enrolment in second LMTX Phase III clinical trial for Alzheimer's disease

TauRx Therapeutics Ltd has achieved its target enrolment of 700 subjects with mild Alzheimer's disease into the second of its two Phase III clinical trials of LMTX, a tau aggregation inhibitor, for the treatment of Alzheimer's disease.

22 Sep 2014

Finding suggests that retina acts as type of window to the brain

Researchers at the Gladstone Institutes and University of California, San Francisco have shown that a loss of cells in the retina is one of the earliest signs of frontotemporal dementia (FTD) in people with a genetic risk for the disorder-even before any changes appear in their behavior.

25 Aug 2014

Increasing clearance of ALS misfolded protein from neurons improves their survival

In work supported by The ALS Association, researchers have shown that increasing the clearance of misfolded protein from neurons improves their survival. The study was published today in the journal Nature Chemical Biology.

30 Jun 2014

New research implicates that RNA processing is central to ALS disease process

In work supported by The ALS Association, researchers have discovered a new ALS-causing gene and have linked its function to that of another prominent disease gene. The study was published in the journal Nature Neuroscience.

1 Apr 2014

Study offers new avenue to pursue in quest for desperately needed treatments for ALS

St. Jude Children's Research Hospital scientists led a study showing that mutations in a gene responsible for amyotrophic lateral sclerosis (ALS) disrupt the RNA transport system in nerve cells.

6 Feb 2014

Researcher finds possible way to unravel misfolded proteins by reprogramming yeast protein

At the heart of brain diseases such as amyotrophic lateral sclerosis, Alzheimer's disease, and Parkinson's disease is protein misfolding, in which distorted proteins are unable to perform their normal functions. At present, there is no known way to reverse protein misfolding.

17 Jan 2014

Research findings point to possible new strategy for treating ALS

Amyotrophic lateral sclerosis, better known as Lou Gehrig's disease, is a devastating illness that gradually robs sufferers of muscle strength and eventually causes a lethal, full-body paralysis. The only drug available to treat the disease extends life spans by a meager three months on average.

17 Dec 2013

Three Penn Medicine researchers awarded grants for neuromuscular disease research

The Muscular Dystrophy Association has awarded research grants totaling over $1,000,000 to three Penn Medicine researchers: James Shorter, PhD, associate professor of Biochemistry and Biophysics; Hansell Stedman, MD, associate professor of Surgery; and Lee Sweeney, PhD, director of the Center for Orphan Disease Research and Therapy.

9 Oct 2013

Researchers identify possible treatment strategy for ALS and other neurodegenerative disorders

Researchers have tied mutations in a gene that causes amyotrophic lateral sclerosis and other neurodegenerative disorders to the toxic buildup of certain proteins and related molecules in cells, including neurons. The research, published recently in the scientific journal Cell, offers a new approach for developing treatments against these devastating diseases.

29 Aug 2013

Penn researchers find ALS disease progresses in a stepwise, sequential pattern

The motor neuron disease Amyotrophic lateral sclerosis (ALS), also known as Lou Gehrig's Disease, progresses in a stepwise, sequential pattern which can be classified into four distinct stages, report pathologists with the Perelman School of Medicine at the University of Pennsylvania in the Annals of Neurology.

20 Jun 2013

Transposons increase in abundance and activity in brains as they age

As the body ages, the physical effects are notable; wrinkles in the skin appear, physical exertion becomes harder. But there are also less visible processes going on. Inside aging brains there is another phenomenon at work, which may contribute to age-related brain defects.

9 Apr 2013