Between clinically affected and healthy sheep, no differences were found in the protein levels of mGluR1, while phospholipase

Cβ1 expression in terminally ill R428 sheep was increased in some brain areas but decreased in others. Adenyl cyclase 1 and A1R levels were significantly lower in various brain areas of affected sheep. No abnormal biochemical expression levels of these markers were found in preclinically infected sheep. Conclusions: These findings point towards an involvement of mGluR1 and A1R downstream pathways in natural scrapie. While classical prion disease lesions and neuromodulatory responses converge in some affected regions, they do not do so in others suggesting that there are independent regulatory

factors for distinct degenerative and neuroprotective responses. “
“Since the first description of the classical presentation of progressive supranuclear palsy (PSP) in 1963, now known as Richardson’s syndrome (PSP-RS), several distinct clinical syndromes have been associated with PSP-tau pathology. Like other neurodegenerative disorders, the severity and distribution of phosphorylated tau pathology are closely associated with the clinical heterogeneity of PSP variants. PSP with corticobasal syndrome presentation (PSP-CBS) was reported to have more tau load in the mid-frontal and inferior-parietal cortices buy Rapamycin than in PSP-RS. However, it is uncertain if differences exist in the distribution of tau pathology in other brain regions or if the overall tau load is increased in the brains of PSP-CBS. We sought

to compare the clinical and pathological features of PSP-CBS and PSP-RS including quantitative assessment of tau load in 15 cortical, basal ganglia and cerebellar regions. In addition to the similar age Myosin of onset and disease duration, we demonstrated that the overall severity of tau pathology was the same between PSP-CBS and PSP-RS. We identified that there was a shift of tau burden towards the cortical regions away from the basal ganglia; supporting the notion that PSP-CBS is a ‘cortical’ PSP variant. PSP-CBS also had less severe neuronal loss in the dorsolateral and ventrolateral subregions of the substantia nigra and more severe microglial response in the corticospinal tract than in PSP-RS; however, neuronal loss in subthalamic nucleus was equally severe in both groups. A better understanding of the factors that influence the selective pathological vulnerability in different PSP variants will provide further insights into the neurodegenerative process underlying tauopathies. “
“Y. Chiba, S. Takei, N. Kawamura, Y. Kawaguchi, K. Sasaki, S. Hasegawa-Ishii, A. Furukawa, M. Hosokawa and A.