New prion protein discovery may shed light on mad-cow disease

Canadian scientists have discovered a new prion protein, a breakthrough that could both help clarify how prion diseases like Creutzfeldt-Jakob or mad-cow disease devastate the brain as well as offer fresh insights into brain function in other neurodegenerative diseases.

London, August 27 : Canadian scientists have discovered a new prion protein, a breakthrough that could both help clarify how prion diseases like Creutzfeldt-Jakob or mad-cow disease devastate the brain as well as offer fresh insights into brain function in other neurodegenerative diseases.

Prion diseases wipe out the brain when an imperfectly folded version of a prion protein called PrP begins converting normal PrP, which usually protects the brain, into the misfolded variety.

Studies, so far, had suspected that other types of prion might exist in the brain, but none had ever been identified till now, reports NewScientist.com.

Now, Joel Watts from the University of Toronto in Canada and his colleagues report that they have isolated a prion protein called 'Shadoo', which is found in the brain cells of mice.

The team suggests that this protein, which shares structural similarities with a key region of the healthy version of PrP, appears to protect neurons.

While examining healthy mouse brains for traces of Shadoo, Watts' team found the protein mainly in areas where PrP was absent, suggesting that it complements PrP's neuroprotective role.

When the researchers infected the mice with misfolded PrP, Shadoo disappeared, implying that a lack of this protein contributes to the damage done by the disease.

Watts is now investigating whether Shadoo can also be converted into a misfolded version, and he hopes that a better understanding of this question might help identify new drug targets for treating prion diseases.

The researchers have reported their findings in an article in the Embo Journal.

ANI