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Scrapie

MedGen UID:
19903
•Concept ID:
C0036457
•
Disease or Syndrome
Synonym: Rida
 
Monarch Initiative: MONDO:0006961

Definition

A fatal disease of the nervous system in sheep and goats, characterized by pruritus, debility, and locomotor incoordination. It is caused by proteinaceous infectious particles called prions. [from MONDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVScrapie

Professional guidelines

PubMed

Strain variation in treatment and prevention of human prion diseases.
Ellett LJ, Revill ZT, Koo YQ, Lawson VA
Prog Mol Biol Transl Sci 2020;175:121-145. Epub 2020 Sep 11 doi: 10.1016/bs.pmbts.2020.08.006. PMID: 32958230
Novel strain properties distinguishing sporadic prion diseases sharing prion protein genotype and prion type.
Cracco L, Notari S, Cali I, Sy MS, Chen SG, Cohen ML, Ghetti B, Appleby BS, Zou WQ, Caughey B, Safar JG, Gambetti P
Sci Rep 2017 Jan 16;7:38280. doi: 10.1038/srep38280. PMID: 28091514Free PMC Article
The prion/lipid hypothesis--further evidence to support the molecular basis for transmissible spongiform encephalopathy risk assessment.
Gale P
J Appl Microbiol 2007 Dec;103(6):2033-45. doi: 10.1111/j.1365-2672.2007.03411.x. PMID: 18045387

Recent clinical studies

Etiology

Prion Diseases.
Whitechurch BC, Welton JM, Collins SJ, Lawson VA
Adv Neurobiol 2017;15:335-364. doi: 10.1007/978-3-319-57193-5_13. PMID: 28674988
Immunotherapy in prion disease.
Roettger Y, Du Y, Bacher M, Zerr I, Dodel R, Bach JP
Nat Rev Neurol 2013 Feb;9(2):98-105. Epub 2012 Dec 18 doi: 10.1038/nrneurol.2012.258. PMID: 23247613
Prion diseases.
Johnson RT
Lancet Neurol 2005 Oct;4(10):635-42. doi: 10.1016/S1474-4422(05)70192-7. PMID: 16168932
Bovine spongiform encephalopathy.
Prince MJ, Bailey JA, Barrowman PR, Bishop KJ, Campbell GR, Wood JM
Rev Sci Tech 2003 Apr;22(1):37-60. doi: 10.20506/rst.22.1.1389. PMID: 12793773
Prions.
Prusiner SB
Proc Natl Acad Sci U S A 1998 Nov 10;95(23):13363-83. doi: 10.1073/pnas.95.23.13363. PMID: 9811807Free PMC Article

Diagnosis

Prion therapeutics: Lessons from the past.
Shim KH, Sharma N, An SSA
Prion 2022 Dec;16(1):265-294. doi: 10.1080/19336896.2022.2153551. PMID: 36515657Free PMC Article
Human transmissible spongiform encephalopathies: historic view.
Asher DM, Gregori L
Handb Clin Neurol 2018;153:1-17. doi: 10.1016/B978-0-444-63945-5.00001-5. PMID: 29887130
Scrapie, CWD, and Transmissible Mink Encephalopathy.
Mathiason CK
Prog Mol Biol Transl Sci 2017;150:267-292. Epub 2017 Aug 10 doi: 10.1016/bs.pmbts.2017.07.009. PMID: 28838664
Subclinical prion infection.
Hill AF, Collinge J
Trends Microbiol 2003 Dec;11(12):578-84. doi: 10.1016/j.tim.2003.10.007. PMID: 14659690
Scrapie.
Hunter N
Mol Biotechnol 1998 Jun;9(3):225-34. doi: 10.1007/BF02915795. PMID: 9718582

Therapy

Oral vaccination as a potential strategy to manage chronic wasting disease in wild cervid populations.
Napper S, Schatzl HM
Front Immunol 2023;14:1156451. Epub 2023 Apr 14 doi: 10.3389/fimmu.2023.1156451. PMID: 37122761Free PMC Article
Prion therapeutics: Lessons from the past.
Shim KH, Sharma N, An SSA
Prion 2022 Dec;16(1):265-294. doi: 10.1080/19336896.2022.2153551. PMID: 36515657Free PMC Article
Molecular neurology of prion disease.
Collinge J
J Neurol Neurosurg Psychiatry 2005 Jul;76(7):906-19. doi: 10.1136/jnnp.2004.048660. PMID: 15965195Free PMC Article
Bovine spongiform encephalopathy.
Prince MJ, Bailey JA, Barrowman PR, Bishop KJ, Campbell GR, Wood JM
Rev Sci Tech 2003 Apr;22(1):37-60. doi: 10.20506/rst.22.1.1389. PMID: 12793773
Amyloidosis.
Cohen AS, Jones LA
Curr Opin Rheumatol 1991 Feb;3(1):125-38. doi: 10.1097/00002281-199102000-00018. PMID: 2043438

Prognosis

Classic and atypical scrapie - a genetic perspective.
Goldmann W
Handb Clin Neurol 2018;153:111-120. doi: 10.1016/B978-0-444-63945-5.00006-4. PMID: 29887131
Immunotherapy in prion disease.
Roettger Y, Du Y, Bacher M, Zerr I, Dodel R, Bach JP
Nat Rev Neurol 2013 Feb;9(2):98-105. Epub 2012 Dec 18 doi: 10.1038/nrneurol.2012.258. PMID: 23247613
Prion diseases.
Johnson RT
Lancet Neurol 2005 Oct;4(10):635-42. doi: 10.1016/S1474-4422(05)70192-7. PMID: 16168932
Amyloidosis.
Cohen AS, Jones LA
Curr Opin Rheumatol 1991 Feb;3(1):125-38. doi: 10.1097/00002281-199102000-00018. PMID: 2043438
Creutzfeldt-Jakob disease and scrapie prions.
Prusiner SB
Alzheimer Dis Assoc Disord 1989 Spring-Summer;3(1-2):52-78. doi: 10.1097/00002093-198903010-00007. PMID: 2568118

Clinical prediction guides

Classic and atypical scrapie - a genetic perspective.
Goldmann W
Handb Clin Neurol 2018;153:111-120. doi: 10.1016/B978-0-444-63945-5.00006-4. PMID: 29887131
Prions: Beyond a Single Protein.
Das AS, Zou WQ
Clin Microbiol Rev 2016 Jul;29(3):633-58. doi: 10.1128/CMR.00046-15. PMID: 27226089Free PMC Article
The stress of prion disease.
Mays CE, Soto C
Brain Res 2016 Oct 1;1648(Pt B):553-560. Epub 2016 Apr 6 doi: 10.1016/j.brainres.2016.04.009. PMID: 27060771
Prion diseases.
Johnson RT
Lancet Neurol 2005 Oct;4(10):635-42. doi: 10.1016/S1474-4422(05)70192-7. PMID: 16168932
Human prion diseases.
Haltia M
Ann Med 2000 Oct;32(7):493-500. doi: 10.3109/07853890009002025. PMID: 11087170

Recent systematic reviews

A systematic review of prion therapeutics in experimental models.
Trevitt CR, Collinge J
Brain 2006 Sep;129(Pt 9):2241-65. Epub 2006 Jul 1 doi: 10.1093/brain/awl150. PMID: 16816391

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