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View article: Susceptibility of Human Prion Protein to Conversion by Chronic Wasting Disease Prions
Susceptibility of Human Prion Protein to Conversion by Chronic Wasting Disease Prions Open
Chronic wasting disease (CWD) is a contagious and fatal neurodegenerative disease and a serious animal health issue for deer and elk in North America. The identification of the first cases of CWD among free-ranging reindeer and moose in Eu…
View article: Rapid amplification of prions from variant Creutzfeldt–Jakob disease cerebrospinal fluid
Rapid amplification of prions from variant Creutzfeldt–Jakob disease cerebrospinal fluid Open
Human prion diseases constitute a group of infectious and invariably fatal neurodegenerative disorders associated with misfolding of the prion protein. Variant Creutzfeldt–Jakob disease (vCJD) is a zoonotic prion disease linked to oral exp…
View article: Human stem cell–derived astrocytes replicate human prions in a <i>PRNP</i> genotype–dependent manner
Human stem cell–derived astrocytes replicate human prions in a <i>PRNP</i> genotype–dependent manner Open
Prions are infectious agents that cause neurodegenerative diseases such as Creutzfeldt–Jakob disease (CJD). The absence of a human cell culture model that replicates human prions has hampered prion disease research for decades. In this pap…
View article: Distribution and Quantitative Estimates of Variant Creutzfeldt-Jakob Disease Prions in Tissues of Clinical and Asymptomatic Patients
Distribution and Quantitative Estimates of Variant Creutzfeldt-Jakob Disease Prions in Tissues of Clinical and Asymptomatic Patients Open
In the United-Kingdom, ≈1 of 2,000 persons could be infected with variant Creutzfeldt-Jakob disease (vCJD). Therefore, risk of transmission of vCJD by medical procedures remains a major concern for public health authorities. In this study,…
View article: UK Iatrogenic Creutzfeldt–Jakob disease: investigating human prion transmission across genotypic barriers using human tissue-based and molecular approaches
UK Iatrogenic Creutzfeldt–Jakob disease: investigating human prion transmission across genotypic barriers using human tissue-based and molecular approaches Open
Creutzfeldt-Jakob disease (CJD) is the prototypic human prion disease that occurs most commonly in sporadic and genetic forms, but it is also transmissible and can be acquired through medical procedures, resulting in iatrogenic CJD (iCJD).…
View article: Relative Abundance of apoE and Aβ1–42 Associated with Abnormal Prion Protein Differs between Creutzfeldt-Jakob Disease Subtypes
Relative Abundance of apoE and Aβ1–42 Associated with Abnormal Prion Protein Differs between Creutzfeldt-Jakob Disease Subtypes Open
Aggregated and protease-resistant mammalian prion protein (PrPSc) is the primary protein component of infectious prions. Enriched PrPSc preparations are often used to study the mechanisms that underly prion disease. However, most enrichmen…
View article: The Distribution of Prion Protein Allotypes Differs Between Sporadic and Iatrogenic Creutzfeldt-Jakob Disease Patients
The Distribution of Prion Protein Allotypes Differs Between Sporadic and Iatrogenic Creutzfeldt-Jakob Disease Patients Open
Sporadic Creutzfeldt-Jakob disease (sCJD) is the most prevalent of the human prion diseases, which are fatal and transmissible neurodegenerative diseases caused by the infectious prion protein (PrP(Sc)). The origin of sCJD is unknown, alth…