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Prions Disease: Creutzfeldt-Jakob's Disease
Transcript of Prions Disease: Creutzfeldt-Jakob's Disease
Diagnosis of Classical (sporadic) CJD
Key Concept 2: The Mystery of Prion Diseases
Prusiner's hypothesis has recently been shown to be viable
Genetic mutation not transmitted genetically (except for FFI)
Nearly indestructible by normal means of sterilization
Unknown phyhsiological function of PRP
1. Considering the virtual indestructibility of defective PRP genes, should extra regulations on hospital sanitation be applied when dealing with prions contaminated materials?
2. Direct injection of prions disease, human or animal, causes the disease incubation to begin in the injected specimen. How does this affect the concept of a species barrier?
3. What challenges do researchers face when researching CJD, considering the accelerated decline of the patients?
Prions Disease: Creutzfeldt-Jakob's Disease
PRP (Protease-resistant protein gene)
Median age of death: 68 years
Symptoms: Personality changes, anxiety, dementia, insomnia, depression
90% die within a year of diagnosis
1 in a million people contract CJD a year
1. The Transmission and Diagnosis of CJD
2. Mystery of Prions disease
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Tschampa, H.J., K. Kallenberg, H.A. Kretzschmar, B. Meissner, M. Knauth, H. Urbach, and I. Zerr. "Pattern of Cortical Changes in Sporadic Creutzfeldt-Jakob Disease." American Journal of Neuroradiology 28.6 (2007): 1114-118. American Journal of Neuroradiology. Web. 13 Oct. 2013.
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Young, Choi Pyo. "PrPSc Complexity in Different Forms of Creutzfeldt-Jakob Disease Identified Using Biochemical Approaches." Diss. The University of Edinburgh, 2010. Abstract. (n.d.): n. pag. Edinburgh Research Archive. Web. 12 Oct. 2013.
Forms of Human Prion Disease
Fatal Familial Insomnia
Bovine spongiform encephalopathy (BSE)
aka Mad Cow Disease
PRP gene found in cattle shares two biochemical features with human PRP gene
Connections to Other Prion Diseases
Though the function of the PRP gene is unknown, would it be advisable to knock out the PRP gene in human embryos to lessen their slight chance of developing CJD? Explain why or why not. Would it be more understandable in fatal familial insomnia?