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Proteomics in protein misfolding diseases
Clinical Chemistry and Laboratory Medicine, 2009Protein misfolding and deposition as amyloid, with consequent tissue damage, plays a key role in the group of diseases generically termed amyloidoses. In the systemic forms, amyloid deposition is widespread and causes severe dysfunction of vital organs.
STOPPINI, MONICA +7 more
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Trashing misfolded membrane proteins
Science, 2014A branch of the endoplasmic reticulum–associated protein degradation system degrades inner nuclear membrane proteins in yeast.
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Protein Misfolding: Optional Barriers, Misfolded Intermediates, and Pathway Heterogeneity
Journal of Molecular Biology, 2004To investigate the character and role of misfolded intermediates in protein folding, a recombinant cytochrome c without the normally blocking histidine to heme misligation was studied. Folding remains heterogeneous as in the wild-type protein. Half of the population folds relatively rapidly to the native state in a two-state manner.
Mallela M G, Krishna +2 more
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Protein Misfolding Cyclic Amplification
2012Prion diseases are caused by a conformational conversion of the cellular prion protein (PrPC) to a pathological conformer (PrPSc). The “prion-only” hypothesis suggests that PrPSc is the infectious agent that propagates the disease acting as a template for the conversion of PrPC.
Moda F., Pritzkow S., Soto C.
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Autophagy and the Metabolism of Misfolding Protein
2019Autophagy is a major intracellular degradative process that delivers cytoplasmic materials to the lysosome for degradation. An increasing number of studies on the physiological and pathological roles of autophagy in a variety of autophagy knockout models and human diseases were carried out.
Chao, Cheng, Zhen-Guo, Liu
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Protein misfolding, evolution and disease
Trends in Biochemical Sciences, 1999I acknowledge very valuable discussions on this article with John Ellis and Carol Robinson. I am grateful to Jose Jiminez and Helen Saibil for providing Figure 3Figure 3 and to Adam Rostom for producing Figure 1Figure 1. This paper is a contribution from the Oxford Centre for Molecular Sciences, which is funded by the BBSRC, EPSRC and MRC. The research
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The folding and misfolding of multidomain proteins
Molecular Aspects of MedicineProtein folding represents a vital process for any living organism. While significant insights have been gained from studying single-domain proteins, our current knowledge on the folding mechanisms of multidomain proteins remains relatively limited, primarily due to their inherent complexity.
Stefano Gianni, Maurizio Brunori
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Mathematics of protein pathological misfolding
Mathematical Biosciences, 2007"Protein folding is defined as a process by which a polypeptide chain performs a search in conformational space with the objective of achieving the so-called native conformation to global free-energy minimum under a given set of physiochemical conditions of the medium." Misfolding then, is the process by which this objective is not achieved.
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Protein misfolding and prion diseases
Journal of Molecular Biology, 1999The prion diseases provide an intriguing connection between protein folding and neurodegenerative disease. In this review, I explore that importance of protein folding and misfolding in the prion diseases. Thermodynamic and kinetic models are examined in an effort to understand infectious, inherited and sporadic forms of these diseases.
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Mechanisms and pathology of protein misfolding and aggregation
Nature Reviews Molecular Cell Biology, 2023Nikolaos Louros +2 more
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