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The Misfolding of Proteins

2020
Protein folding is considered strongly linked with genetics. The deeper understanding of the mechanisms that allow the folding of proteins is expected to lead to advances in the pharmaceutical sector. The shape of a protein dictates its biological activity, and any exceptions from its natural form can lead to diseases such as Alzheimer's, Parkinson's ...
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Protein degradation, aggregation, and misfolding

Movement Disorders, 2010
AbstractThe cellular surveillance systems guarantee proper removal of altered components from inside cells. Alterations of these systems in neurons have been proposed to be involved in the pathogenesis of different neurodegenerative disorders. In this review, we comment on the advances in our current understanding of how changes in the intracellular ...
Ana Maria, Cuervo   +2 more
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Protein misfolding and disease: the case of prion disorders [PDF]

open access: yesCellular and Molecular Life Sciences, 2003
Recent findings strongly support the hypothesis that diverse human disorders, including the most common neurodegenerative diseases, arise from misfolding and aggregation of an underlying protein.
Claudio Soto
exaly   +2 more sources

Protein folding and misfolding

Nature, 2003
The manner in which a newly synthesized chain of amino acids transforms itself into a perfectly folded protein depends both on the intrinsic properties of the amino-acid sequence and on multiple contributing influences from the crowded cellular milieu.
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Protein Misfolding and Human Disease

Annual Review of Genomics and Human Genetics, 2006
Protein misfolding is a common event in living cells. In young and healthy cells, the misfolded protein load is disposed of by protein quality control (PQC) systems. In aging cells and in cells from certain individuals with genetic diseases, the load may overwhelm the PQC capacity, resulting in accumulation of misfolded proteins.
Niels, Gregersen   +3 more
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Principles of Protein Misfolding

2008
Publisher Summary This chapter discusses the principles of protein misfolding. The process of incorrect folding is called “protein misfolding.” It is associated with a number of pathological states in humans, collectively termed “protein-misfolding diseases.” Among these protein-misfolding diseases, amyloid diseases are characterized by the presence ...
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Protein misfolding in hemophilia

Over the past century, the development of advancements in hemophilia therapy has experienced unprecedented success, starting with virally contaminated blood product infusions and progressing to safe plasma-derived and recombinant factor replacements, non-factor based rebalancing agents, long-lasting gene therapies, and moving forward into an era of ...
Bilgimol, Chumappumkal Joseph   +4 more
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Proteomics in protein misfolding diseases

Clinical Chemistry and Laboratory Medicine, 2009
Protein 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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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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Autophagy and the Metabolism of Misfolding Protein

2019
Autophagy 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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