National Institute of Animal Health (NIAH)

Topics in Animal Health Research 2012

16. Brilliant Blue G250 prevents abnormal prion accumulation in vitro and in vivo

Japanese

Prion diseases are fatal neurodegenerative disorders with no effective therapy currently available. We have focused on Brilliant Blue G (BBG), which possesses a chemical structure expected to confer anti-prion activity, and examined its inhibitory effect on the accumulation of pathogenic isoforms of prion protein (PrPres) in a cellular and a mouse model of prion disease, in order to determine its therapeutic potential. BBG prevented PrPres accumulation in infected MG20 microglial cells at 50% inhibitory concentrations (IC50) of 14.6 µM. Administration of BBG in vivo also reduced PrPres accumulation in the brains of mice with prion disease. However, it did not appear to alleviate the disease progression compared to the vehicle-treated controls. These results provide novel insights into the pathophysiology of prion diseases and have important implications for the treatment.
(Prion Diseases Research Center)

References:

Iwamaru Y. et al. (2012) PLoS ONE 7(5): e37896

Centers・Institutes