ALS disease mechanism discovered

September 4, 2018, Umea University
Clumps of SOD1 protein (coloured brown) in the nerve cell that handle the body's motor functions. The image comes from an ALS patient lacking mutation in SOD1. This indicates that the protein is involved in the disease mechanism of ALS even if no mutation occurs in the protein. Credit: Karin Forsberg

A mechanism for amyotrophic lateral sclerosis (ALS) development has been discovered at Umeå University, Sweden. The researchers report that proteins with a defect structure spread the deformation to other proteins. The discovery could result in novel pharmaceutical developments in the future.

"We've been able to identify two different types of aggregates with different structures and propagation abilities. One type gave rise to a more aggressive disease progression, which shows that these aggregates are the driving force in the development of ALS," says Johan Bergh, doctoral student at the Department of Medical Biosciences at Umeå University.

Together with the ALS group at Umeå University, Johan Bergh has developed a method of investigating protein aggregates formed in ALS, Amyotrophic lateral sclerosis. With this new method, it has then been possible to identify the particular protein aggregates that are driving in the emergence of ALS.

The protein that has been targeted is superoxid dismutas-1, SOD1. It has long been known that mutations in that protein can cause ALS. The goal of the research team was to investigate the way in which the protein contributes to the disease. In several diseases afflicting the nervous system, such as in Alzheimer's and Parkinson's Disease, new studies show that some proteins assume an abberant structure. Misfolded proteins aggregate and provoke other proteins of the same kind to assume the same structure. In this way, the disease spreads step by step into the nervous system.

"Using the new method, we have shown and confirmed through animal models that the development of ALS follows the same principle as for other severe nervous disorders. Protein aggregates function as a template that healthy proteins stick to and cause the disease to spread," says Johan Bergh.

In animal models, agregates of the SOD1 protein from animals, as well as humans, have been shown to induce ALS disease.

Amyotrophic lateral sclerosis, ALS, is a which afflicts approximately 250 people annually in Sweden. Although the disease has been known for over 100 years, there is still only one medicine with a disease delaying effect available in Sweden.

"Through our new method, I hope that in the future, drugs will be developed specifically aimed at attacking these protein aggregates. Hopefully, research teams focusing on similar diseases will adopt the . However, we are in an early phase, and developing drugs is a long-term process," says Johan Bergh.

Explore further: Powerful molecules provide new findings about Huntington's disease

Related Stories

Powerful molecules provide new findings about Huntington's disease

August 21, 2018
Researchers at Lund University in Sweden have discovered a direct link between the protein aggregation in nerve cells that is typical for neurodegenerative diseases, and the regulation of gene expression in Huntington's disease. ...

Aggregated protein in nerve cells can cause ALS

May 4, 2016
Persons with the serious disorder ALS, can have a genetic mutation that causes the protein SOD1 to aggregate in motor neurons in the brain and spinal cord. Researchers at Umeå University have discovered that, when injected ...

Study identifies chaperone protein implicated in Parkinson's disease

August 13, 2018
Reduced levels of a chaperone protein might have implications for the development and progression of neurodegenerative diseases such as Parkinson's disease and Lewy body dementia, according to new research from investigators ...

Researchers reveal unusual chemistry of protein with role in neurodegenerative disorders

July 27, 2017
A common feature of neurodegenerative diseases is the formation of permanent tangles of insoluble proteins in cells. The beta-amyloid plaques found in people with Alzheimer's disease and the inclusion bodies in motor neurons ...

Recommended for you

Study of protein 'trafficker' provides insight into autism and other brain disorders

September 22, 2018
In the brain, as in business, connections are everything. To maintain cellular associates, the outer surface of a neuron, its membrane, must express particular proteins—proverbial hands that reach out and greet nearby cells. ...

Breast milk may be best for premature babies' brain development

September 21, 2018
Babies born before their due date show better brain development when fed breast milk rather than formula, a study has found.

Early warning sign of psychosis detected

September 21, 2018
Brains of people at risk of psychosis exhibit a pattern that can help predict whether they will go on to develop full-fledged schizophrenia, a new Yale-led study shows. The findings could help doctors begin early intervention ...

White matter repair and traumatic brain injury

September 20, 2018
Traumatic brain injury (TBI) is a leading cause of death and disability in the U.S., contributing to about 30 percent of all injury deaths, according to the CDC. TBI causes damage to both white and gray matter in the brain, ...

Gut branches of vagus nerve essential components of brain's reward and motivation system

September 20, 2018
A novel gut-to-brain neural circuit establishes the vagus nerve as an essential component of the brain system that regulates reward and motivation, according to research conducted at the Icahn School of Medicine at Mount ...

Genomic dark matter activity connects Parkinson's and psychiatric diseases

September 20, 2018
Dopamine neurons are located in the midbrain, but their tendril-like axons can branch far into the higher cortical areas, influencing how we move and how we feel. New genetic evidence has revealed that these specialized cells ...

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.