Is there more to prion protein than mad cow disease?

Sep 30, 2008

Prion protein, a form of protein that triggers BSE, is associated with other brain diseases in cattle, raising the possibility of a significant increase in the range of prion disease. Publishing their findings in the open access journal BMC Veterinary Research, scientists have detected changes in the production and accumulation of the prion protein in the brains of cattle with a rare neurodegenerative disorder.

Martin Jeffrey of the Veterinary Laboratories Agency led a research team that tested 15 brains of cattle with idiopathic brainstem neuronal chromatolysis and hippocampal sclerosis (IBNC). They are the first group to show that the brains of animals with this disease accumulate prion protein (PrP), the protein that misfolds to cause BSE and which, when transmitted to humans through the food chain, can cause the deadly Variant Creutzfeldt-Jakob disease

IBNC is a rare neurological disease of adult cattle. It was first characterised in 1988 following laboratory tests from cattle suspected of having BSE. Although IBNC has some clinical similarities to BSE, the brains of affected cattle do not have the neuronal vacuolation (lesions) typical of BSE.

Further laboratory tests suggest that the misfolded form of PrP, which accumulates in the brains of BSE cases, is not present in IBNC cases. Commercial BSE testing kits did not detect the telltale, BSE-inducing form of PrP either. However, the presence of increased levels of PrP was detected.

"We've shown for the first time that prion protein is somehow involved in IBNC," says Jeffrey, "In this disease, there is an association with abnormally high levels of a prion protein in the brain but clearly this PrP is in a different form to that involved in BSE and CJD. This may have implications for diagnosis and recognition of typical forms of BSE as well as the related diseases in sheep, deer and in man.

Source: BioMed Central

Explore further: Life-prolonging protein could inhibit ageing diseases

Related Stories

EPA says first day of oil spill spent 'planning'

9 hours ago

On the afternoon of the largest coastal oil spill in California in 25 years, graduate student Natalie Phares quickly organized a volunteer bucket brigade to clean a beach north of Santa Barbara.

Insect mating behavior has lessons for drones

9 hours ago

Male moths locate females by navigating along the latter's pheromone (odor) plume, often flying hundreds of meters to do so. Two strategies are involved to accomplish this: males must find the outer envelope ...

Recommended for you

Life-prolonging protein could inhibit ageing diseases

May 29, 2015

Researchers have found a molecule that plays a key link between dietary restriction and longevity in mammals. This discovery may lead to the development of new therapies to inhibit age-related diseases.

How sleep helps us learn and memorize

May 28, 2015

Sleep is important for long lasting memories, particularly during this exam season. Research publishing in PLOS Computational Biology suggests that sleeping triggers the synapses in our brain to both streng ...

User comments : 0

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.