Content deleted Content added
m cite repair; |
Fixed “prp (protease resistan prion protein)” into its correct form, prion protein, VPSPr proves that PRP-Sc can be protease sensitive and not always protein resistant Tags: Mobile edit Mobile web edit |
||
Line 2:
{{Distinguish|Prion|text=[[Prion|prions]], infectious forms of proteins which have so far been observed in almost all instances to be forms of PRNP, but need not be}}
{{Infobox_gene}}
'''''PRNP''''' ('''prion protein''') is the human [[gene]] encoding for the major [[prion]] protein '''PrP''' (
The protein can exist in multiple [[isoforms]]: the normal '''PrP<sup>C</sup>''' form, and the [[protease]]-resistant form designated '''PrP<sup>Res</sup>''' such as the disease-causing '''PrP<sup>Sc(scrapie)</sup>''' and an isoform located in [[mitochondria]]. The [[Protein folding#Protein misfolding and neurodegenerative disease|misfolded]] version PrP<sup>Sc</sup> is associated with a variety of [[cognitive disorder]]s and [[Neurodegeneration|neurodegenerative]] diseases such as in animals: [[ovine]] [[scrapie]], [[bovine spongiform encephalopathy]] (BSE, mad cow disease), [[feline spongiform encephalopathy]], [[transmissible mink encephalopathy]] (TME), [[exotic ungulate encephalopathy]], [[chronic wasting disease]] (CWD) which affects [[cervids]]; and in humans: [[Creutzfeldt–Jakob disease]] (CJD), [[fatal familial insomnia]] (FFI), [[Gerstmann–Sträussler–Scheinker syndrome]] (GSS), [[Kuru (disease)|kuru]], and [[variant Creutzfeldt–Jakob disease]] (vCJD). Similarities exist between kuru, thought to be due to human ingestion of diseased individuals, and vCJD, thought to be due to human ingestion of BSE-tainted cattle products.
|