Nav: Home

A step closer to identifying cause of a blinding disease

May 23, 2019

Leber hereditary optic neuropathy (LHON) is an inherited form of vision loss that causes people to have trouble with their colour vision and difficulty seeing in the centre of their visual field. Due to the founder effect from the filles du roi, there is a disproportionate preponderance of a particular LHON mutation among the French-Canadian population.

Until recently, researchers believed the cause of LHON was related to the size of the fibers that connect the eye to the brain, with small fibers being more sensitive. However, it wasn't clear how exactly the two were connected.

A recent study, led by researchers at McGill University and published in the journal Scientific Reports, offers an important step in unlocking the mystery of LHON's cause. The researchers had previously showed that the cells that connect the eye to the brain were sensitive to a certain free radical, known as "superoxide," and hypothesized that the presence of too much superoxide was likely the cause of LHON.

"We speculated that superoxide spreading among the fibers of those cells was the cause of the specific clinical features of LHON. We realized that we could test this hypothesis by computer simulation," explains Dr. Leonard Levin, Chair of the Department of Ophthalmology and Visual Sciences at McGill University's Faculty of Medicine and the paper's senior author.

Dr. Levin collaborated with Dr. Razek Coussa, who did the work while a resident in the Department of Ophthalmology and Visual Sciences, and Pooya Merat, a PhD candidate in the Department of Electrical and Computer Engineering at McGill, who provided the computing power needed to render the simulations possible. To validate their findings, the team compared the results of their simulations to pathology specimens and visual fields from patients acquired through colleagues at UCLA, and the researchers were surprised by how well the two were aligned.

While this finding represents an important step, Dr. Levin notes that work remains to be done. "We need to confirm the role of superoxide in spreading the damage in actual cells. If we can do so, this could go a long way towards helping us develop a treatment for LHON."
-end-
"Propagation and Selectivity of Axonal Loss in Leber Hereditary Optic Neuropathy," by Razek Coussa et al. was published online in Scientific Reports on April 30. https://doi.org/10.1038/s41598-019-43180-z

McGill University

Related Brain Articles:

Study describes changes to structural brain networks after radiotherapy for brain tumors
Researchers compared the thickness of brain cortex in patients with brain tumors before and after radiation therapy was applied and found significant dose-dependent changes in the structural properties of cortical neural networks, at both the local and global level.
Blue Brain team discovers a multi-dimensional universe in brain networks
Using a sophisticated type of mathematics in a way that it has never been used before in neuroscience, a team from the Blue Brain Project has uncovered a universe of multi-dimensional geometrical structures and spaces within the networks of the brain.
New brain mapping tool produces higher resolution data during brain surgery
Researchers have developed a new device to map the brain during surgery and distinguish between healthy and diseased tissues.
Newborn baby brain scans will help scientists track brain development
Scientists have today published ground-breaking scans of newborn babies' brains which researchers from all over the world can download and use to study how the human brain develops.
New test may quickly identify mild traumatic brain injury with underlying brain damage
A new test using peripheral vision reaction time could lead to earlier diagnosis and more effective treatment of mild traumatic brain injury, often referred to as a concussion.
More Brain News and Brain Current Events

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

Anthropomorphic
Do animals grieve? Do they have language or consciousness? For a long time, scientists resisted the urge to look for human qualities in animals. This hour, TED speakers explore how that is changing. Guests include biological anthropologist Barbara King, dolphin researcher Denise Herzing, primatologist Frans de Waal, and ecologist Carl Safina.
Now Playing: Science for the People

#534 Bacteria are Coming for Your OJ
What makes breakfast, breakfast? Well, according to every movie and TV show we've ever seen, a big glass of orange juice is basically required. But our morning grapefruit might be in danger. Why? Citrus greening, a bacteria carried by a bug, has infected 90% of the citrus groves in Florida. It's coming for your OJ. We'll talk with University of Maryland plant virologist Anne Simon about ways to stop the citrus killer, and with science writer and journalist Maryn McKenna about why throwing antibiotics at the problem is probably not the solution. Related links: A Review of the Citrus Greening...