Prions are lethal mammalian pathogens composed of aggregated conformational isomers of
a host-encoded glycoprotein and which appear to lack nucleic acids. Their unique biology …

Prions are unusual proteinaceous infectious agents that are typically associated with a class
of fatal degenerative diseases of the mammalian brain. However, the discovery of fungal …

Most mutations in the dystrophin gene create a frameshift or a stop in the mRNA and are
associated with severe Duchenne muscular dystrophy. Exon skipping that naturally occurs …

Variant Creutzfeldt-Jakob disease (vCJD) is a unique and highly distinctive
clinicopathological and molecular phenotype of human prion disease associated with …

A conformational transition of normal cellular prion protein (PrPC) to its pathogenic form
(PrPSc) is believed to be a central event in the transmission of the devastating neurological …

Prion diseases are believed to propagate by the mechanism involving self-perpetuating
conformational conversion of the normal form of the prion protein, PrP C, to the misfolded …

Two normally occurring polymorphisms of the human PRNP gene, methionine (M)/valine (V)
at codon 129 and glutamic acid (E)/lysine (K) at codon 219, can affect the susceptibility to …

Prion diseases in mammals are caused by a conformational transition of the cellular prion
protein from its native conformation (PrPC) to a pathological isoform called “prion protein …

Inherited prion diseases are neurodegenerative disorders caused by autosomal dominant
mutations in the human prion protein gene (PRNP). Kindred with inherited prion disease can …

Conformational transitions of the cellular form of the prion protein, PrP C, into an infectious
isoform, PrP Sc, are considered to be central events in the progression of fatal …