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Different reactive profiles of calmodulin in the CSF samples of Chinese patients of four types of genetic prion diseases. [PDF]
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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 ...
James A Mastrianni
exaly +3 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 ...
James A Mastrianni
exaly +3 more sources
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
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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
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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
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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