Prion disease research has contributed much toward understanding other neurodegenerative diseases, including recent demonstrations that Alzheimer's disease (AD) and other neurodegenerative diseases are prion-like. Prion-like diseases involve the spread of degeneration between individuals and/or among cells or tissues via template directed misfolding, wherein misfolded protein conformers propaga...

No remedies for prion disease have been established, and the conversion of normal to abnormal prion protein, a key event in prion disease, is still unclear. Here we found that substances in beetle grub hemolymph, after they were browned by aging for a month or heating for hours, reduced abnormal prion protein (PrP) levels in RML prion-infected cells. Active anti-prion components in the hemolymp...

A crucial tenet of the prion hypothesis is that misfolding of the prion protein (PrP) induced by mutations associated with familial prion disease is, in an otherwise normal mammalian brain, sufficient to generate the infectious agent. Yet this has never been demonstrated. We engineered knockin mice to express a PrP mutation associated with a distinct human prion disease, fatal familial insomnia...

If the attacking force has a vaccine before attack, will inflict crippling damage on enemy. Thus, virus emerges as potential danger since they can be used in development of such weapons. The reported neurotropic strain influenza A (IAV) induced conversion normal prion protein (PrPC) into infectious (PrPSc) well formation prions bring out type prion/virus unprecedented pathogens that cause fatal...

Inherited human prion diseases, such as fatal familial insomnia (FFI) and familial Creutzfeldt-Jakob disease (fCJD), are associated with autosomal dominant mutations in the human prion protein gene PRNP and accumulation of PrPSc, an abnormal isomer of the normal host protein PrPC, in the brain of affected individuals. PrPSc is the principal component of the transmissible neurotoxic prion agent....

OBJECTIVE To identify cases of inherited prion diseases in Britain and to assess their phenotypic features. DESIGN Screening study of patients suspected clinically to have Creutzfeldt-Jakob disease and other neurodegenerative diseases by prion protein gene analysis. SETTING Biochemical research department. SUBJECTS Patients suspected to have Creutzfeldt-Jakob disease and other neurodegene...

creutzfeldt-jacob disease is the most common form of prion diseases, which have become public health problems in the last two decades because of the high number of reported cases of mad cow disease in great britain and other countries. creutzfeldt-jacob disease is a fatal situation with known cardinal clinical features including progressive memory loss and myoclonic seizure disorder. in this re...

Prion diseases are transmissible neurodegenerative disorders which affect a range of mammalian species. In humans they can be inherited and sporadic as well as acquired by exposure to human prions. Prions appear to be composed principally of a conformational isomer of host-encoded prion protein and propagate by recruitment of cellular prion protein. Recent evidence argues that prion protein can...

BACKGROUND Gerstmann-Sträussler-Scheinker disease is a rare form of prion disease. OBJECTIVE To determine the prion mutation in a 51-year-old man without a family history of neurologic disease who died from Gerstmann-Sträussler-Scheinker disease. PATIENT AND METHODS The patient was a 51-year-old man who died after a 9-year illness characterized by dementia and eventually ataxia. Neuropathol...

Organotypic cerebellar slices represent a suitable model for characterizing and manipulating prion replication in complex cell environments. Organotypic slices recapitulate prion pathology and are amenable to drug testing in the absence of a blood-brain-barrier. So far, the cellular and subcellular distribution of disease-specific prion protein in organotypic slices is unclear. Here we report t...