Infectious Prions Detected in Skin of Patients With Neurodegenerative Creutzfeldt-Jakob Disease

MedicalResearch.com Interview with:

The brain of one patient who died from sporadic Creutzfeldt-Jacob disease (sCJD) appears nearly identical to the brain of a mouse inoculated with infectious prions taken from the skin of patients who died from sCJD.

The brain of one patient who died from sporadic Creutzfeldt-Jacob disease (sCJD) appears nearly identical to the brain of a mouse inoculated with infectious prions taken from the skin of patients who died from sCJD.
Case Western Reserve University

 

Byron Caughey, Ph.D.
Senior Investigator
Chief, TSE/prion Biochemistry Section
Laboratory of Persistent Viral Diseases
NIH/NIAID Rocky Mountain Laboratories
Hamilton, MT 

 

 

MedicalResearch.com: Would you briefly explain what is meant by Creutzfeldt-Jakob disease?

Response: Creutzfeldt-Jakob disease (CJD) is an incurable—and ultimately fatal—transmissible, neurodegenerative disorder in the family of prion diseases. Prion diseases can be found in many mammalian species and are due to the conversion of normally harmless prion protein molecules into abnormally folded, aggregated and self-propagating clusters and filaments in the brain. The accumulation of these clusters has been associated with tissue damage that often leaves dying neurons and microscopic sponge-like holes in the brain. In the sporadic and genetic forms of CJD this pathogenic process appears to arise spontaneously in the patient.

However, the transfer of the prion protein aggregates from a Creutzfeldt-Jakob disease patient into another human or experimental animal can initiate the pathogenic process in the recipient. These infectious forms of prion protein are called prions. Human prion diseases include fatal insomnia; kuru; Gerstmann-Straussler-Scheinker syndrome; and variant, familial and sporadic CJD. Sporadic CJD is the most common human prion disease, affecting about one in one million people annually worldwide. Other prion diseases include scrapie in sheep; chronic wasting disease in deer, elk and moose; and bovine spongiform encephalopathy (BSE), or mad cow disease, in cattle.

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Creutzfeldt-Jakob Disease: Cerebrospinal Fluid Total and Phosphorylated Tau

MedicalResearch.com Interview with:
Tobias Skillbäck, MD
Clinical Neurochemistry Laboratory
Institute of Neuroscience and Physiology
Department of Neurochemistry, Sahlgrenska Academy
University of Gothenburg Mölndal, Sweden

MedicalResearch.com: What are the main findings of the study?

Dr. Skillbäck: There were two main findings in this study.

First; Levels of t-tau and the T-tau/P-tau ratio in CSF of CJD (Creutzfeldt-Jakob Disease) patients are markedly increased, as compared to patients with Alzheimer’s disease and other dementias, and they  are high enough to distinguish CJD against these important differential diagnoses.

Secondly, levels of these biomarkers tend to increase rapidly with disease progress in Creutzfeldt-Jakob Disease. This trend could not be observed for Alzheimer’s disease and other dementias, and could also be used to clinically distinguish CJD and indicates that repeated CSF measurements might be of value if a clinical suspicion of Creutzfeldt-Jakob Diseaseis present.
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