Researchers find mechanism that could prevent or treat deadly peroxisome diseases

January 7, 2010

University of Alberta medical researchers have made a major breakthrough in understanding a group of deadly disorders that includes the disease made famous in the movie Lorenzo's Oil.

Because this group of diseases is inherited, the discovery could help in screening carriers and lead to prevention or an effective treatment.

Richard Rachubinski, in the Faculty of Medicine & Dentistry, is an expert on structures in cells called peroxisomes which are involved in breaking down fatty acids. They are vital for humans. Babies born with a peroxizome disorder do not typically survive longer than a year because of impaired metabolism.

In his latest study, Rachubinski found another clue in the search to understand the peroxisome and the disorders caused by its malfunction. He and his team discovered that a protein family thought to be only involved in the early stages of making peroxisomes is actually crucial in ensuring peroxisomes transfer into other cells after they divide. All cells of the body must have peroxisomes to survive. These proteins are found in every living being, so they provide a universal mechanism not only for how peroxisomes are made but also for how peroxisomes are maintained in to keep them alive as they divide.

One peroxisome disorder is adrenoleukodystrophy, or ALD, the disease a boy named Lorenzo Odone suffered from. His parents' fight for a cure was made into the movie.

Rachubinski's findings have been published in the .

Explore further: Molecular motors cooperate in moving cellular cargo, study shows

Related Stories

Recommended for you

How the finch changes its tune

August 3, 2015

Like top musicians, songbirds train from a young age to weed out errors and trim variability from their songs, ultimately becoming consistent and reliable performers. But as with human musicians, even the best are not machines. ...

Cow embryos reveal new type of chromosome chimera

May 27, 2016

I've often wondered what happens between the time an egg is fertilized and the time the ball of cells that it becomes nestles into the uterine lining. It's a period that we know very little about, a black box of developmental ...

Shaving time to test antidotes for nerve agents

February 29, 2016

Imagine you wanted to know how much energy it took to bike up a mountain, but couldn't finish the ride to the peak yourself. So, to get the total energy required, you and a team of friends strap energy meters to your bikes ...

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.