Nav: Home

University of Guelph study uncovers cause of cell death in Parkinson's disease

February 26, 2018

A University of Guelph researcher has discovered one of the factors behind nerve cell death in Parkinson's disease, unlocking the potential for treatment to slow the progression of this fatal neurodegenerative disorder.

Prof. Scott Ryan has found that cardiolipin, a molecule inside nerve cells, helps ensure that a protein called alpha-synuclein folds properly. Misfolding of this protein leads to protein deposits that are the hallmark of Parkinson's disease.

These deposits are toxic to nerve cells that control voluntary movement. When too many of these deposits accumulate, nerve cells die.

"Identifying the crucial role cardiolipin plays in keeping these proteins functional means cardiolipin may represent a new target for development of therapies against Parkinson's disease," said Ryan, a professor in U of G's Department of Molecular and Cellular Biology. "Currently there are no treatments that stop nerve cells from dying."

Parkinson's disease is the most common degenerative movement disorder in Canada, affecting about 100,000 people.

Published in the journal Nature Communications, the study used stem cells collected from people with the disease. Ryan's research team studied how nerve cells try to cope with misfolded alpha-synuclein.

"We thought if we can better understand how cells normally fold alpha-synuclein, we may be able to exploit that process to dissolve these aggregates and slow the spread of the disease," he said.

Funded by Parkinson Canada, the study revealed that, inside cells, alpha-synuclein binds to mitochondria, where cardiolipin resides. Cells use mitochondria to generate energy and drive metabolism.

Normally, cardiolipin in mitochondria pulls synuclein out of toxic protein deposits and refolds it into a non-toxic shape.

The U of G researchers found that, in people with Parkinson's disease, this process is overwhelmed over time and mitochondria are ultimately destroyed, said Ryan.

"As a result, the cells slowly die. Based on this finding, we now have a better understanding of why nerve cells die in Parkinson's disease and how we might be able to intervene."

He said understanding cardiolipin's role in protein refolding may help in creating a drug or therapy to slow progression of the disease.

"The hope is that we will be able to rescue locomotor deficits in an animal model. It's a big step towards treating the cause of this disease."
-end-


University of Guelph

Related Mitochondria Articles:

Mitochondria-targeted antioxidant SkQ1 helps to treat diabetic wounds
Members of the Faculty of Biology and A.N. Belozersky Institute of Physico-Chemical Biology, a unit of the Lomonosov Moscow State University, have tested on a mouse model a mitochondria-targeted antioxidant, helping to treat diabetic wounds.
Mitochondria targeting anti-tumor compound
Researchers from Kumamoto University in Japan have found that the compound folic acid-conjugated methyl-BETA-cyclodextrin (FA-M-BETA-CyD) has significant antitumor effects on folate receptor-ALPHA-expressing (FR-ALPHA (+)) cancer cells.
Closing the gate to mitochondria
A team of researchers develops a new method that enables the identification of proteins imported into mitochondria.
Elucidated connection between renal failure and 'bad' mitochondria described
Biologists from the A.N. Belozersky Institute of Physico-Chemical Biology, a unit of the Lomonosov Moscow State University suggested the approach to prevent kidney injury after ischemia.
How exercise -- interval training in particular -- helps your mitochondria stave off old age
Researchers have long suspected that the benefits of exercise extend down to the cellular level, but know relatively little about which exercises help cells rebuild key organelles that deteriorate with aging.
Cell disposal faults could contribute to Parkinson's, study finds
A fault with the natural waste disposal system that helps to keep our brain cell 'batteries' healthy may contribute to neurodegenerative disease, a new study has found.
Sex cells evolved to pass on quality mitochondria
Mammals immortalize their genes through eggs and sperm to ensure future generations inherit good quality mitochondria to power the body's cells, according to new UCL research.
Newly identified pathway in mitochondria fuels tumor progression across cancer types
Scientists at The Wistar Institute have identified a novel protein pathway across several types of cancer that controls how tumor cells acquire the energy necessary for movement, invasion and metastasis.
Collapse of mitochondria-associated membrane in ALS
Mitochondria-associated membrane (MAM) is a contacting site of endoplasmic reticulum and mitochondria, and plays a key role in cellular homeostasis.
New research on the muscles of elite athletes: When quality is better than quantity
A Danish-Swedish research team working on a project led by University of Southern Denmark has discovered that muscle endurance is not only determined by the number of mitochondria, but also their structure.

Related Mitochondria Reading:

Best Science Podcasts 2019

We have hand picked the best science podcasts for 2019. Sit back and enjoy new science podcasts updated daily from your favorite science news services and scientists.
Now Playing: TED Radio Hour

Changing The World
What does it take to change the world for the better? This hour, TED speakers explore ideas on activism—what motivates it, why it matters, and how each of us can make a difference. Guests include civil rights activist Ruby Sales, labor leader and civil rights activist Dolores Huerta, author Jeremy Heimans, "craftivist" Sarah Corbett, and designer and futurist Angela Oguntala.
Now Playing: Science for the People

#521 The Curious Life of Krill
Krill may be one of the most abundant forms of life on our planet... but it turns out we don't know that much about them. For a create that underpins a massive ocean ecosystem and lives in our oceans in massive numbers, they're surprisingly difficult to study. We sit down and shine some light on these underappreciated crustaceans with Stephen Nicol, Adjunct Professor at the University of Tasmania, Scientific Advisor to the Association of Responsible Krill Harvesting Companies, and author of the book "The Curious Life of Krill: A Conservation Story from the Bottom of the World".