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{{Distinguish|Prion|text=[[prion]]s, 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}}
'''Major prion protein''' '''(PrP)''', is encoded in the human by the ''PRNP'' [[gene]] also known as '''CD230''' ([[cluster of differentiation]] 230).<ref name = "pmid3755672">{{cite journal | vauthors = Kretzschmar HA, Stowring LE, Westaway D, Stubblebine WH, Prusiner SB, Dearmond SJ | title = Molecular cloning of a human prion protein cDNA | journal = DNA | volume = 5 | issue = 4 | pages = 315–24 | date = August 1986 | pmid = 3755672 | doi = 10.1089/dna.1986.5.315}}</ref><ref name = "pmid3094007">{{cite journal | vauthors = Sparkes RS, Simon M, Cohn VH, Fournier RE, Lem J, Klisak I, Heinzmann C, Blatt C, Lucero M, Mohandas T | title = Assignment of the human and mouse prion protein genes to homologous chromosomes | journal = Proc. Natl. Acad. Sci. U.S.A. | volume = 83 | issue = 19 | pages = 7358–62 | date = October 1986 | pmid = 3094007 | pmc = 386716 | doi = 10.1073/pnas.83.19.7358 | bibcode = 1986PNAS...83.7358S | doi-access = free }}</ref><ref name = "pmid3014653">{{cite journal | vauthors = Liao YC, Lebo RV, Clawson GA, Smuckler EA | title = Human prion protein cDNA: molecular cloning, chromosomal mapping, and biological implications | journal = Science | volume = 233 | issue = 4761 | pages = 364–7 | date = July 1986 | pmid = 3014653 | doi = 10.1126/science.3014653 | bibcode = 1986Sci...233..364L }}</ref><ref name = "pmid2877664">{{cite journal | vauthors = Robakis NK, Devine-Gage EA, Jenkins EC, Kascsak RJ, Brown WT, Krawczun MS, Silverman WP | title = Localization of a human gene homologous to the PrP gene on the p arm of chromosome 20 and detection of PrP-related antigens in normal human brain | journal = Biochem. Biophys. Res. Commun. | volume = 140 | issue = 2 | pages = 758–65 | date = October 1986 | pmid = 2877664 | doi = 10.1016/0006-291X(86)90796-5 }}</ref> Expression of the [[protein]] is most predominant in the [[nervous system]] but occurs in many other tissues throughout the body.<ref name = "pmid11357156">{{cite journal | vauthors = Prusiner SB | title = Shattuck lecture--neurodegenerative diseases and prions | journal = N Engl J Med | volume = 344 | issue = 20 | pages = 1516–26 | year = 2001 | pmid = 11357156 | doi = 10.1056/NEJM200105173442006}}</ref><ref name = "pmid15494743">{{cite journal | vauthors = Weissmann C | title = The state of the prion | journal = Nat Rev Microbiol | volume = 2 | issue = 11 | pages = 861–71 | year = 2004 | pmid = 15494743 | doi = 10.1038/nrmicro1025| s2cid = 20992257 }}</ref><ref name = "review2">{{cite journal | vauthors = Zomosa-Signoret V, Arnaud JD, Fontes P, Alvarez-Martinez MT, Liautard JP | title = Physiological role of the cellular prion protein | journal = Vet. Res. | volume = 39 | issue = 4 | pages = 9 | year = 2008 | pmid = 18073096 | doi = 10.1051/vetres:2007048| url = https://hal.archives-ouvertes.fr/hal-00902908/file/hal-00902908.pdf | doi-access = free }}</ref>
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.
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