小中大
研究prion主要要弄清楚如下一些问题,这些问题有些已经有比较明确的答案,但是大部分都还没搞清楚或存在较大的争论,希望大家多讨论讨论。我希望在以后的贴子中尽我可能把这些问题的最新进展整理出来与大家讨论。
1. What is the nature of the infectious agent?
(1) Is PrPSc identical with the prion?
(2) How does PrPSc promote PrPC conversion into further PrPSc?
(3) Which other proteins assist in this process?
(4) Can we cure prion diseases by interfering with the conversion process?
(5) What are prion strains, molecularly speaking? How are the strain-speci.c properties encoded?
2. How do peripherally administered prions travel to the brain?
(1) Which molecules and cells are involved in neuroinvasion?
(2) Which inhibitory strategies have a realistic chance of succeeding?
3. What are the mechanisms of spongiform neurodegeneration?
(1) Which pathogenetic cascades are activated?
(2) What are the biochemical executioners of brain damage?
4. What is the physiological function of the normal prion protein, PrPC?
(1) PrPC is highly conserved, so it is probably useful.
(2) The Prnp gene was identi.ed in 1985, and Prnp knockout mice in 1992, yet the function of PrPC remains unknown.
(3) None of the phenotypes ascribed to the absence of PrPC has been explained in molecular terms.
(4) Is abnormal function of PrPC involved in prion disease pathogenesis?
5.why do we have prions?