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  Protein Misfolding Diseases

Hartl, F. U. (2017). Protein Misfolding Diseases. Annual Review of Biochemistry, 86, 21-26. doi:10.1146/annurev-biochem-061516-044518.

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 Urheber:
Hartl, F. Ulrich1, Autor           
Affiliations:
1Hartl, Franz-Ulrich / Cellular Biochemistry, Max Planck Institute of Biochemistry, Max Planck Society, ou_1565152              

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Schlagwörter: NUCLEOCYTOPLASMIC TRANSPORT; PROTEOTOXIC STRESS; AGGREGATION; PROTEOSTASIS; HUNTINGTIN; NUCLEARprotein folding; protein misfolding; protein aggregation; amyloid; molecular chaperone; neurodegenerative disease; protein homeostasis; proteostasis;
 Zusammenfassung: The majority of protein molecules must fold into defined three-dimensional structures to acquire functional activity. However, protein chains can adopt a multitude of conformational states, and their biologically active conformation is often only marginally stable. Metastable proteins tend to populate misfolded species that are prone to forming toxic aggregates, including soluble oligomers and fibrillar amyloid deposits, which are linked with neurodegeneration in Alzheimer and Parkinson disease, and many other pathologies. To prevent or regulate protein aggregation, all cells contain an extensive protein homeostasis (or proteostasis) network comprising molecular chaperones and other factors. These defense systems tend to decline during aging, facilitating the manifestation of aggregate deposition diseases. This volume of the Annual Review of Biochemistry contains a set of three articles addressing our current understanding of the structures of pathological protein aggregates and their associated disease mechanisms. These articles also discuss recent insights into the strategies cells have evolved to neutralize toxic aggregates by sequestering them in specific cellular locations.

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Sprache(n): eng - English
 Datum: 2017-04-242017
 Publikationsstatus: Erschienen
 Seiten: -
 Ort, Verlag, Ausgabe: -
 Inhaltsverzeichnis: -
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Titel: Annual Review of Biochemistry
  Alternativer Titel : Annu. Rev. Biochem..
  Alternativer Titel : ANNU REV BIOCHEM
Genre der Quelle: Konferenzband
 Urheber:
Affiliations:
Ort, Verlag, Ausgabe: 4139 EL CAMINO WAY, PO BOX 10139, PALO ALTO, CA 94303-0897 USA : ANNUAL REVIEWS
Seiten: - Band / Heft: 86 Artikelnummer: - Start- / Endseite: 21 - 26 Identifikator: ISSN: 0066-4154
ISBN: 978-0-8243-0886-5

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Titel: Annual Review of Biochemistry
  Alternativer Titel : ANNU REV BIOCHEM
  Alternativer Titel : Annu. Rev. Biochem..
Genre der Quelle: Reihe
 Urheber:
Affiliations:
Ort, Verlag, Ausgabe: -
Seiten: - Band / Heft: 86 Artikelnummer: - Start- / Endseite: - Identifikator: ISSN: 0066-4154