Results 181 to 190 of about 42,402 (225)
Some of the next articles are maybe not open access.
Neurologic Clinics, 2018
Prions diseases are uniformly fatal neurodegenerative diseases that occur in sporadic, genetic, and acquired forms. Acquired prion diseases, caused by infectious transmission, are least common. Most prion diseases are not infectious, but occur spontaneously through misfolding of normal prion proteins or genetic mutations in the prion protein gene ...
Boon Lead, Tee +2 more
openaire +4 more sources
Prions diseases are uniformly fatal neurodegenerative diseases that occur in sporadic, genetic, and acquired forms. Acquired prion diseases, caused by infectious transmission, are least common. Most prion diseases are not infectious, but occur spontaneously through misfolding of normal prion proteins or genetic mutations in the prion protein gene ...
Boon Lead, Tee +2 more
openaire +4 more sources
Current Biology, 1992
There have been remarkably rapid advances in the understanding of prion diseases over the past year. The controversial notion that the transmissible agent may be an abnormal isoform of a host-encoded protein, the prion protein, is now gaining wide acceptance.
J, Collinge, M S, Palmer
openaire +2 more sources
There have been remarkably rapid advances in the understanding of prion diseases over the past year. The controversial notion that the transmissible agent may be an abnormal isoform of a host-encoded protein, the prion protein, is now gaining wide acceptance.
J, Collinge, M S, Palmer
openaire +2 more sources
Prion Diseases and Emerging Prion Diseases
Current Medicinal Chemistry, 2008Transmissible spongiform encephalopathies (TSEs), also called prion diseases, are fatal neurodegenerative disorders. An abnormal isoform of the prion protein (PrP(Sc)) generated by post-translational modification of the cellular prion protein (PrP(C)) is believed to be the main component of this infectious agent.
Takashi, Yokoyama, Shirou, Mohri
openaire +2 more sources
Clinical pediatrics, 1997
Prion diseases are a group of disorders sharing clinical and pathological features. Many of the enigmas of these diseases have now yielded to the concerted effort to understand them and their unusual pathogenesis. The genetic backgrounds of the various diseases are being clarified at an impressive rate, but the cause of sporadic Creutzfeldt-Jakob ...
Shetty, Avinash K., Steele, Russell W.
openaire +7 more sources
Prion diseases are a group of disorders sharing clinical and pathological features. Many of the enigmas of these diseases have now yielded to the concerted effort to understand them and their unusual pathogenesis. The genetic backgrounds of the various diseases are being clarified at an impressive rate, but the cause of sporadic Creutzfeldt-Jakob ...
Shetty, Avinash K., Steele, Russell W.
openaire +7 more sources
Journal of Neurovirology, 2003
Prion diseases are incurable neurodegenerative conditions affecting both animals and humans. They may be sporadic, infectious, or inherited in origin. Human prion diseases include Creutzfeldt-Jakob desease (CJD), Gerstmann-Straussler-Scheinker disease, kuru, and fatal familial insomnia.
Edward, McKintosh +2 more
openaire +2 more sources
Prion diseases are incurable neurodegenerative conditions affecting both animals and humans. They may be sporadic, infectious, or inherited in origin. Human prion diseases include Creutzfeldt-Jakob desease (CJD), Gerstmann-Straussler-Scheinker disease, kuru, and fatal familial insomnia.
Edward, McKintosh +2 more
openaire +2 more sources
Haemophilia, 1998
Summary. Although the nature of the infectious agent causing prion diseases is still debated, several of its molecular characteristics have been clarified in remarkable detail. The transmissibility of bovine spongiform encephalopathy to humans dramatically highlights the need for research focused at interference with prion replication and spread, and ...
C. A. Lee +6 more
openaire +4 more sources
Summary. Although the nature of the infectious agent causing prion diseases is still debated, several of its molecular characteristics have been clarified in remarkable detail. The transmissibility of bovine spongiform encephalopathy to humans dramatically highlights the need for research focused at interference with prion replication and spread, and ...
C. A. Lee +6 more
openaire +4 more sources
Seminars in Neurology, 2019
AbstractPrion diseases are a phenotypically diverse set of disorders characterized by protease-resistant abnormally shaped proteins known as prions. There are three main groups of prion diseases, termed sporadic (Creutzfeldt–Jakob disease [CJD], sporadic fatal insomnia, and variably protease-sensitive prionopathy), genetic (genetic CJD, fatal familial ...
Kelly J, Baldwin, Cynthia M, Correll
openaire +2 more sources
AbstractPrion diseases are a phenotypically diverse set of disorders characterized by protease-resistant abnormally shaped proteins known as prions. There are three main groups of prion diseases, termed sporadic (Creutzfeldt–Jakob disease [CJD], sporadic fatal insomnia, and variably protease-sensitive prionopathy), genetic (genetic CJD, fatal familial ...
Kelly J, Baldwin, Cynthia M, Correll
openaire +2 more sources
The Lancet Neurology, 2005
Prion diseases are degenerative disorders of the nervous system caused by transmissible particles that contain a pathogenic isoform of the prion protein, a normal constituent of cell membranes. The most common human prion disease is Creutzfeldt-Jakob disease (CJD). Most cases are sporadic with unknown mode of transmission, 10-15% of cases are inherited,
openaire +2 more sources
Prion diseases are degenerative disorders of the nervous system caused by transmissible particles that contain a pathogenic isoform of the prion protein, a normal constituent of cell membranes. The most common human prion disease is Creutzfeldt-Jakob disease (CJD). Most cases are sporadic with unknown mode of transmission, 10-15% of cases are inherited,
openaire +2 more sources
Journal of Geriatric Psychiatry and Neurology, 2010
The prion diseases are a family of rare neurodegenerative disorders that result from the accumulation of a misfolded isoform of the prion protein (PrP), a normal constituent of the neuronal membrane. Five subtypes constitute the known human prion diseases; kuru, Creutzfeldt-Jakob disease (CJD), Gerstmann-Sträussler-Scheinker syndrome (GSS), fatal ...
Khalilah, Brown, James A, Mastrianni
openaire +2 more sources
The prion diseases are a family of rare neurodegenerative disorders that result from the accumulation of a misfolded isoform of the prion protein (PrP), a normal constituent of the neuronal membrane. Five subtypes constitute the known human prion diseases; kuru, Creutzfeldt-Jakob disease (CJD), Gerstmann-Sträussler-Scheinker syndrome (GSS), fatal ...
Khalilah, Brown, James A, Mastrianni
openaire +2 more sources
2018
Genetic prion diseases (gPrDs) are caused by autosomal-dominant mutations in the prion protein gene (PRNP). Although the first PRNP mutations identified, and most since, are PRNP missense, octapeptide repeat insertions, deletion and nonsense mutations have now also been shown to cause gPrD. Based on clinicopathologic features of familial disease, gPrDs
Leonel T, Takada +4 more
openaire +2 more sources
Genetic prion diseases (gPrDs) are caused by autosomal-dominant mutations in the prion protein gene (PRNP). Although the first PRNP mutations identified, and most since, are PRNP missense, octapeptide repeat insertions, deletion and nonsense mutations have now also been shown to cause gPrD. Based on clinicopathologic features of familial disease, gPrDs
Leonel T, Takada +4 more
openaire +2 more sources