Over-activity of enzyme HDAC6 exacerbates symptoms of Amyotrophic Lateral Sclerosis

June 26, 2014

Scientists at VIB and KU Leuven have demonstrated in fruit-flies that over-activity of the enzyme HDAC6 in the nerve ends exacerbates the symptoms of the neurodegenerative condition Amyotrophic Lateral Sclerosis (ALS / Lou Gehrig's Disease). Inhibition of this enzyme could offer a protective effect against ALS.

Patrik Verstreken (VIB/KU Leuven): "Nobody wants to suffer from a degenerative condition such as (ALS), in which you lose coordination of all muscles through gradual damage to the . ALS patients are conscious throughout the disease process, including death by suffocation. Any additional insight into the dysfunction of nerve cells and the defective transmission of signals to the muscles paves the way to further targeted ALS research."

Amyotrophic Lateral Sclerosis – a degenerative process

In patients with ALS, the nerve cells that allow for the die off. Synaptic transmission is the transmission of signals to the muscles, which is essential for the coordination of the muscles. ALS falls under the collective term of neurodegenerative conditions, which includes diseases such as Parkinson's disease. It is a progressive, debilitating neurological disease for which there is currently no treatment available. ALS patients are very aware of the entire process. ALS patients often opt for euthanasia in order to avoid suffocating to death as the respiratory muscles also fail in the end.

Over-activity HDAC6

Katarzyna Miskiewicz, Liya Jose and Patrik Verstreken have studied how this type of nerve cell becomes defective in fruit-flies that express a form of ALS. Fruit-flies are a very suitable model organism for neurological research. In the case of ALS, the specific defect is located at the nerve endings, where the investigators discovered an over-activity of the enzyme HDAC6. This over-activity of HDAC6 is a cause of the disrupted synaptic transmission, resulting in a symptomatic exacerbation of the disease. In order to stop this process, it would – in theory – be helpful to combat the over-activation of the enzyme HDAC6 in order to return to "normal" activity of the HDAC6 enzyme.

Not only HDAC6 defect

Although this discovery may provide a possible avenue for symptomatic ALS treatment, it should be noted that the HDAC6 defect is probably one of the many enzyme defects that play a role in ALS. Previously, Patrik Verstreken and Wim Robberecht (VIB/KU Leuven) demonstrated that another that is involved in ALS – Elp3 – acts on the same process at the as HDAC6. Ideally, the various avenues should all be studied in the hope of finding a treatment for ALS in the future.

Explore further: Researchers identify new gene mutation associated with ALS

More information: HDAC6 Is a Bruchpilot Deacetylase that Facilitates Neurotransmitter Release, Cell Reports, www.cell.com/cell-reports/abst … -1247%2814%2900445-8

Related Stories

Researchers identify new gene mutation associated with ALS

April 1, 2014
A research team led by investigators at the National Institute on Aging at the National Institutes of Health has discovered a new gene mutation associated with ALS, amyotrophic lateral sclerosis. The mutation is involved ...

Zebra fish point the way towards new therapies for amyotrophic lateral sclerosis

August 27, 2012
Leuven scientists (VIB/KU Leuven) are using zebrafish as a model in their search for genes that play a role in the mechanism of amyotrophic lateral sclerosis (ALS). As a result, they have identified a molecule that could ...

Researchers discover how ALS spreads

February 18, 2014
A study led by University of British Columbia and Vancouver Coastal Health Research Institute researchers has revealed how the fatal neurodegenerative disease amyotrophic lateral sclerosis (ALS), also known as Lou Gehrig's ...

Blocking metabolic protein improves movement in animals with amyotrophic lateral sclerosis

January 17, 2012
Turning off a protein that helps cells balance energy increases animal mobility and reduces the death of nerve cells that control movement in animal models of amyotrophic lateral sclerosis (ALS), according to a study in the ...

Scientists identify new gene that influences survival in amyotrophic lateral sclerosis

August 26, 2012
A team of scientists, including faculty at the University of Massachusetts Medical School (UMMS), have discovered a gene that influences survival time in amyotrophic lateral sclerosis (ALS, also known as Lou Gehrig's disease). ...

Recommended for you

Researchers find monkey brain structure that decides if viewed objects are new or unidentified

August 18, 2017
A team of researchers working at the University of Tokyo School of Medicine has found what they believe is the part of the monkey brain that decides if something that is being viewed is recognizable. In their paper published ...

Artificial neural networks decode brain activity during performed and imagined movements

August 18, 2017
Artificial intelligence has far outpaced human intelligence in certain tasks. Several groups from the Freiburg excellence cluster BrainLinks-BrainTools led by neuroscientist private lecturer Dr. Tonio Ball are showing how ...

Study of nervous system cells can help to understand degenerative diseases

August 18, 2017
The results of a new study show that many of the genes expressed by microglia differ between humans and mice, which are frequently used as animal models in research on Alzheimer's disease and other neurodegenerative disorders.

How whip-like cell appendages promote bodily fluid flow

August 18, 2017
Researchers at Nagoya University have identified a molecule that enables cell appendages called cilia to beat in a coordinated way to drive the flow of fluid around the brain; this prevents the accumulation of this fluid, ...

Researchers make surprising discovery about how neurons talk to each other

August 17, 2017
Researchers at the University of Pittsburgh have uncovered the mechanism by which neurons keep up with the demands of repeatedly sending signals to other neurons. The new findings, made in fruit flies and mice, challenge ...

Neurons involved in learning, memory preservation less stable, more flexible than once thought

August 17, 2017
The human brain has a region of cells responsible for linking sensory cues to actions and behaviors and cataloging the link as a memory. Cells that form these links have been deemed highly stable and fixed.

0 comments

Please sign in to add a comment. Registration is free, and takes less than a minute. Read more

Click here to reset your password.
Sign in to get notified via email when new comments are made.