Human prion diseases and the prion protein – what is the current state of knowledge?

Prion diseases and the prion protein are only partially understood so far in many aspects. This explains the continued research on this topic, calling for an overview on the current state of knowledge. The main objective of the present review article is to provide a comprehensive up-to-date presenta...
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Autor*in:	Nafe Reinhold [verfasserIn] Arendt Christophe T. [verfasserIn] Hattingen Elke [verfasserIn]

Format:	E-Artikel
Sprache:	Englisch

Erschienen:	2023

Schlagwörter:	creutzfeldt–jakob disease variably protease-sensitive prionopathy fatal familial insomnia gerstmann–sträussler–scheinker disease kuru

Übergeordnetes Werk:	In: Translational Neuroscience - De Gruyter, 2015, 14(2023), 1, Seite 60-83
Übergeordnetes Werk:	volume:14 ; year:2023 ; number:1 ; pages:60-83

Links:	Link aufrufen Link aufrufen Link aufrufen Journal toc

DOI / URN:	10.1515/tnsci-2022-0315

Katalog-ID:	DOAJ101306865

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topic_title	RC321-571 Human prion diseases and the prion protein – what is the current state of knowledge? creutzfeldt–jakob disease variably protease-sensitive prionopathy fatal familial insomnia gerstmann–sträussler–scheinker disease kuru
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title	Human prion diseases and the prion protein – what is the current state of knowledge?
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title_sort	human prion diseases and the prion protein – what is the current state of knowledge?
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title_auth	Human prion diseases and the prion protein – what is the current state of knowledge?
abstract	Prion diseases and the prion protein are only partially understood so far in many aspects. This explains the continued research on this topic, calling for an overview on the current state of knowledge. The main objective of the present review article is to provide a comprehensive up-to-date presentation of all major features of human prion diseases bridging the gap between basic research and clinical aspects. Starting with the prion protein, current insights concerning its physiological functions and the process of pathological conversion will be highlighted. Diagnostic, molecular, and clinical aspects of all human prion diseases will be discussed, including information concerning rare diseases like prion-associated amyloidoses and Huntington disease-like 1, as well as the question about a potential human threat due to the transmission of prions from prion diseases of other species such as chronic wasting disease. Finally, recent attempts to develop future therapeutic strategies will be addressed.
abstractGer	Prion diseases and the prion protein are only partially understood so far in many aspects. This explains the continued research on this topic, calling for an overview on the current state of knowledge. The main objective of the present review article is to provide a comprehensive up-to-date presentation of all major features of human prion diseases bridging the gap between basic research and clinical aspects. Starting with the prion protein, current insights concerning its physiological functions and the process of pathological conversion will be highlighted. Diagnostic, molecular, and clinical aspects of all human prion diseases will be discussed, including information concerning rare diseases like prion-associated amyloidoses and Huntington disease-like 1, as well as the question about a potential human threat due to the transmission of prions from prion diseases of other species such as chronic wasting disease. Finally, recent attempts to develop future therapeutic strategies will be addressed.
abstract_unstemmed	Prion diseases and the prion protein are only partially understood so far in many aspects. This explains the continued research on this topic, calling for an overview on the current state of knowledge. The main objective of the present review article is to provide a comprehensive up-to-date presentation of all major features of human prion diseases bridging the gap between basic research and clinical aspects. Starting with the prion protein, current insights concerning its physiological functions and the process of pathological conversion will be highlighted. Diagnostic, molecular, and clinical aspects of all human prion diseases will be discussed, including information concerning rare diseases like prion-associated amyloidoses and Huntington disease-like 1, as well as the question about a potential human threat due to the transmission of prions from prion diseases of other species such as chronic wasting disease. Finally, recent attempts to develop future therapeutic strategies will be addressed.
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