Reduced expression of the presynaptic co-chaperone cysteine string protein alpha (CSPα) does not exacerbate experimentally-induced ME7 prion disease.

摘要: Infection of mice with the ME7 prion agent results in well-characterised neuropathological changes, which includes vacuolation, neurodegeneration and synaptic degeneration. Presynaptic dysfunction degeneration is apparent through progressive reduction vesicle proteins eventual loss synapses. Cysteine string protein alpha (CSPα), regulates refolding pathways at synapse, exhibits an early decline during chronic implicating it as a mediator disease mechanisms. CSPα null develop neuronal disruption integrity presynaptic function. In this study, we investigated whether reduced expression would exacerbate disease. Wild type (+/+) heterozygous (+/−) mice, express about ∼50% CSPα, were used distinct genetic background on to impose +/+ +/ − inoculated brain homogenate from either normal mouse (NBH) or displayed clinical signs (ME7). Behavioural tests, western blotting immunohistochemistry, resolve key elements dysfunction, assess effect tests revealed no change progression ME7–CSPα +/− animals compared animals. addition, accumulation misfolded PrPSc, diseased associated gliosis not different. Thus, misfolding events that generate lead are unlikely be mediated by decrease CSPα.