publication . Article . 2017

Prion-like disorders and Transmissible Spongiform Encephalopathies: An overview of the mechanistic features that are shared by the various disease-related misfolded proteins

Joaquín Castilla; Jorge Moreno; Vanesa Venegas; Hasier Eraña;
Open Access
  • Published: 01 Feb 2017
Abstract
Prion diseases or Transmissible Spongiform Encephalopathies (TSEs) are a group of fatal neurodegenerative disorders affecting several mammalian species. Its causative agent, disease-associated prion protein (PrPd), is a self-propagating β-sheet rich aberrant conformation of the cellular prion protein (PrPC) with neurotoxic and aggregation-prone properties, capable of inducing misfolding of PrPC molecules. PrPd is the major constituent of prions and, most importantly, is the first known example of a protein with infectious attributes. It has been suggested that similar molecular mechanisms could be shared by other proteins implicated in diseases such as Alzheimer...
Subjects
Medical Subject Headings: animal diseasesnervous system diseases
free text keywords: Biophysics, Cell Biology, Biochemistry, Molecular Biology, Virology, Protein folding, A protein, Biology, Protein Misfolding Disorder, Prion protein, Prion strain, Amyotrophic lateral sclerosis, medicine.disease, medicine, Genetics, Disease, Protein aggregation
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