A newly discovered type of prion an aberrant protein implicated in the transmission of progressive neurodegenerative diseases like mad cow disease and Creutzfeldt-Jakob Disease has been linked to the development of a rare but fatal brain disease called Multiple System Atrophy (MSA). This has the potential to force us to rethink the way we conceive of our relation to the environment, particularly when it comes to our diets.
WHAT IS MULTIPLE SYSTEM ATROPY?
MSA is a neurodegenerative illness that progressively attacks the autonomic nervous system, hampering involuntary body functions like breathing and digestion, as well as voluntary movements. When cells in the brain and spinal cord lose their functions and die, they lead to symptoms characteristic of the disease: fainting spells, abnormal heart rate, muscle tremors, rigidity and difficulty with speech. Symptoms tend to appear around 50 years of age, and worsen rapidly leading to incapacitation and eventual death due to respiratory or cardiac issues. Though it is a rare disease affecting 15,000-50,000 Americans, it has no cure and its cause is unknown. It shares a lot of its symptoms with Parkinson’s disease, which further confounds diagnosis.
WE NOW KNOW THAT A NEW TYPE OF PRION CAUSES MSA
Scientists have discovered that a type of infectious protein called alpha-synuclein prion is responsible for the transmission of MSA. They looked at 14 human brain samples obtained from people with MSA. Mice were used to test whether MSA could be transmitted to them when they come in contact with MSA tissue. All 14 of the brain samples caused mice to develop neurodegeneration. This type of transmission was not noted for Parkinsonian brains, thereby proving that different agents cause Parkinson’s disease and MSA.
HOW DO PRIONS INFECT BRAINS?
The transmissible, almost viral nature of prions has been studied previously in Creutzfeldt-Jakob Disease, in which prions called PrP in the brain undergo misfolding transformations, which changes their shape and function. These can then infect other PrP molecules, causing them to misfold, thereby propagating the loss of function. Prions have the distinctive ability to form sticky patches on the brain, eventually killing off healthy cells. The authors of the study recommend extreme caution and stringent safety protocols when dealing with patients with prion disorders. Since proteins, unlike viruses or bacteria, cannot be disinfected by common sterilization regimens, usage of different surgical equipment for prion diseases and other neurodegenerative conditions is highly advised.
The researchers of this study have tied up with Daiichi Sankyo as part of a project to develop potential therapies for prion diseases.
TIRED BRAIN CELLS MAY LEAD TO PARKINSON’S DISEASE
