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Prion proteins meet protein quality control

Trends in Cell Biology, 2003
Recent work on transmissible spongiform encephalopathies (TSEs) suggests a role for protein quality-control mechanisms in both prion protein aggregation and pathogenesis. Cytosolic accumulation of prion protein seems to be neurotoxic and might occur when proteasome function is compromised and quality control is overwhelmed. These findings are discussed
Derek E, Dimcheff   +2 more
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Protein Quality Control in Mitochondria

Journal of Biochemistry, 2009
Mitochondria are crucial for both life and death of eukaryotic cells. Compromised mitochondrial integrity has severe cellular consequences and is linked to senescence and neurodegenerative disorders in humans. To maintain the functionality of proteins in mitochondria, quality-control mechanisms including signal transduction pathways counteracting ...
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Quality Control: Proteins and Organelles

Biochemistry (Moscow), 2002
This review summarizes materials on the mechanisms of intracellular degradation of proteins whose topogenesis is disturbed at one stage or another. Chaperone and proteolytic systems involved in this process in the endoplasmic reticulum, mitochondria, and chloroplasts of eucaryotic cells as well as those in distinct subcellular compartments of ...
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Target Selection during Protein Quality Control

Trends in Biochemical Sciences, 2016
Protein quality control (QC) pathways survey the cellular proteome to selectively recognize and degrade faulty proteins whose accumulation can lead to various diseases. Recognition of the occasional aberrant protein among an abundant sea of similar normal counterparts poses a considerable challenge to the cell.
Sichen, Shao, Ramanujan S, Hegde
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Protein Quality Control in Neurodegenerative Disease

2012
The accumulation of misfolded proteins is a common feature of many neurodegenerative diseases. These observations suggest a potential link between these disorders and protein quality control, a collection of cellular pathways that sense damage to proteins and facilitate their turnover. Consistent with this idea, activation of quality control components,
Jason E, Gestwicki, Dan, Garza
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Nucleolus: A Protein Quality Control Compartment

Trends in Biochemical Sciences, 2019
Frottin et al. uncovered a role for the nucleolus as a key quality control compartment that regulates misfolded nuclear proteins. This nonmembrane compartment achieves this by forming liquid-like protein condensates that aid protein refolding in a heat-shock protein (Hsp)70-dependent manner.
Rekulapally, Pavani, Suresh, S N
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Coping with Protein Quality Control Failure

Annual Review of Cell and Developmental Biology, 2017
Cells and organisms have evolved numerous mechanisms to cope with and to adapt to unexpected challenges and harsh conditions. Proteins are essential to perform the vast majority of cellular and organismal functions. To maintain a healthy proteome, cells rely on a network of factors and pathways collectively known as protein quality control (PQC ...
Esther, Pilla   +2 more
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New developments for protein quality control

Science, 2017
A unique enzyme promotes degradation of misfolded proteins and differentiation of red blood ...
Randolph Y, Hampton   +1 more
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Protein quality control at the mitochondrion

Essays in Biochemistry, 2016
Mitochondria are essential constituents of a eukaryotic cell by supplying ATP and contributing to many mayor metabolic processes. As endosymbiotic organelles, they represent a cellular subcompartment exhibiting many autonomous functions, most importantly containing a complete endogenous machinery responsible for protein expression, folding and ...
Wolfgang, Voos   +3 more
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Protein Quality Control in the Bacterial Periplasm

Annual Review of Microbiology, 2011
Protein quality control involves sensing and treatment of defective or incomplete protein structures. Misfolded or mislocalized proteins trigger dedicated signal transduction cascades that upregulate the production of protein quality-control factors. Corresponding proteases and chaperones either degrade or repair damaged proteins, thereby reducing the
Merdanovic, Melisa   +4 more
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