Myelin is a fatty substance that forms a sheath for nerve fibers, known as axons, and facilitates the conduction of nerve signals. Its loss through disease causes impairment of sensation, movement, cognition and other functions, depending on which nerves are affected.

The new study arose from a mysterious affliction of pregnant cats. A company testing the effects on growth and development in cats using diets that had been irradiated reported that some cats developed severe neurological dysfunction, including movement disorders, vision loss and paralysis. Taken off the diet, the cats recovered slowly, but eventually all lost functions were restored.

"After being on the diet for three to four months, the pregnant cats started to develop progressive neurological disease," says Duncan, a professor of medical sciences at the UW-Madison School of Veterinary Medicine and an authority on demyelinating diseases. "Cats put back on a normal diet recovered. It's a very puzzling demyelinating disease."

The afflicted cats were shown to have severe and widely distributed demyelination of the central nervous system, according to Duncan. And while the neurological symptoms exhibited by the cats are similar to those experienced by humans with demyelination disorders, the malady does not seem to be like any of the known myelin-related diseases of humans.

In cats removed from the diet, recovery was slow, but all of the previously demyelinated axons became remyelinated. The restored myelin sheaths, however, were not as thick as healthy myelin, Duncan notes.

"It's not normal, but from a physiological standpoint, the thin myelin membrane restores function," he says. "It's doing what it is supposed to do."

Knowing that the central nervous system retains the ability to forge new myelin sheaths anywhere the nerves themselves are preserved provides strong support for the idea that if myelin can be restored in diseases such as multiple sclerosis, it may be possible for patients to regain lost or impaired functions: "The key thing is that it absolutely confirms the notion that remyelinating strategies are clinically important," Duncan says.

The exact cause of the neurological affliction in the cats on the experimental diet is unknown, says Duncan, who was not involved in the original study of diet.

"We think it is extremely unlikely that [irradiated food] could become a human health problem," Duncan explains. "We think it is species specific. It's important to note these cats were fed a diet of irradiated food for a period of time."

In addition to Duncan, authors of the new PNAS study include Alexandra Brower of the Wisconsin Veterinary Diagnostic Laboratory; Yoichi Kondo and Ronald Schultz of the UW-Madison School of Veterinary Medicine; and Joseph Curlee Jr. of Harlan Laboratories in Madison.

Source
University Communications
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http://www.medicalnewstoday.com/articles/144348.php
 
article kindly supplied by Juan Carlos Baiges

 Article Date: 31 Mar 2009 - 6:00 PDT

The legacy of Marie Schlau: literature to help cure Friedreich's Ataxia

If you feel like reading an unputdownable novel while collaborating with a just and solidary cause, "The Legacy of Marie Schlau" is your book! 100% of all funds raised will be dedicated to medical research to find a cure for Friedreich's Ataxia, a neurodegenerative disease that affects mostly young people, shortening their life expectancy and confining them to a wheelchair.

The life of Marie Schlau, a German Jewish girl born in 1833 hides great unsolved mysteries: accidents, disappearances, enigmas, unknown diagnoses, disturbing murders, love, tenderness, greed, lies, death ... alternatively a different story unfolds every time and takes us closer to the present. Thus, there are two parallel stories unravelling, each in a different age and place, which surprisingly converge in a revelatory chapter.

Paperback and Kindle versions for "The legacy of Marie Schlau" available for sale at Amazon now!

https://www.amazon.com/Legacy-Marie-Schlau-collective-Friedreichs-ebook/dp/B01N28AFWZ

 

Research projects currently being financed by BabelFAmily

Currently, BabelFAmily is financing two promising research projects aimed at finding a cure for Friedreich's Ataxia. Whenever you make a donation to us or purchase a copy of "The legacy of Marie Schlau", this is where all funds raised will be devoted to:

1) Gene Therapy for Friedreich's Ataxia research project:

https://www.irbbarcelona.org/en/news/international-patient-advocates-partner-to-fund-spanish-gene-therapy-project-to-treat

The project is the result of an initiative of Spanish people affected by this rare disease who are grouped in GENEFA in collaboration with the Spanish Federation of Ataxias and the BabelFAmily. The Friedreich’s Ataxia Research Alliance (FARA), one of the main patients’ associations in the United States now joins the endeavour.

2) Frataxin delivery research project:

https://www.irbbarcelona.org/en/news/new-research-front-to-tackle-friedreichs-ataxia
The associations of patients and families Babel Family and the Asociación Granadina de la Ataxia de Friedreich (ASOGAF) channel 80,000 euros of their donations (50% from each organisation) into a new 18-month project at the Institute for Research in Biomedicine (IRB Barcelona). The project specifically aims to complete a step necessary in order to move towards a future frataxin replacement therapy for the brain, where the reduction of this protein causes the most damage in patients with Friedreich’s Ataxia.

The study is headed by Ernest Giralt, head of the Peptides and Proteins Lab, who has many years of experience and is a recognised expert in peptide chemistry and new systems of through which to delivery drugs to the brain, such as peptide shuttles—molecules that have the capacity to carry the drug across the barrier that surrounds and protects the brain. Since the lab started its relation with these patients’ associations in 2013*, it has been developing another two projects into Friedrich’s Ataxia.

 

 

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