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Showing 1 to 20 of 55 for “"Protein quality control"”.
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The Systems Biology of the Protein Quality Control
… protease, is the main powerhouse for protein degradation in all eukaryotic cells. Proteostasis is maintained by the ubiquitin-proteasome system (UPS) targeting aberrant proteins whose accumulation and aggregation are hallmarks of aging and neurodegenerative disorders. Previous studies …
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Protein quality control processes in the yeast golgi
Thesis (Ph. D.)--Massachusetts Institute of Technology, Dept. of Biology, 1997.
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Protein quality control in the mammalian endoplasmic reticulum
Quality control is an important part of protein biogenesis. Aberrant proteins must be destroyed before they aggregate and cause deleterious effects. Failure to do so can result in cell death or malfunction and, ultimately, disease. Quality control involves the recognition of misfolded proteins and …
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DYSREGULATION of PROTEIN QUALITY CONTROL IMPAIRS FUNCTION of PRIMARY CARDIOMYOCYTES
… have a rapid turnover and impair of gap junction quality control impacts cell-to-cell communication; resulting in electrical conduction abnormalities and arrhythmogenesis. Therefore, understanding the underlying mechanism the quality control of mitochondria and gap junctions profoundly contributes …
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Investigating dysregulation in protein quality control mechanisms in senescent cells
… pathways and mechanisms which protect cellular protein homeostasis, including synthesis, folding, trafficking and degradation of proteins. Proteostasis loss is implicated in both normal ageing and diseases of ageing in humans, although which mechanisms are disrupted and how is currently unclear. …
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The Phase Behavior of Ubqln Proteins and Implications for Protein Quality Control
… autophagy are essential pathways for maintaining protein quality control (PQC) in cells. Misfolded proteins and large aggregates are cleared by UPS and autophagy signaled by ubiquitin (Ub) or polyubiquitin (polyUb) chains. Shuttle proteins facilitate cargo transporting by interacting with both …
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Redox Sensing By Yeast Hsp70 Facilitates Modulation of Protein Quality Control and The Cytoprotective Response
… of these aggregates is damage sustained to proteins by oxidative stress. Cellular protein homeostasis (proteostasis) relies on the ubiquitous Hsp70 chaperone family. Hsp70 activity has been previously shown to be modulated by modification of two key cysteines in the ATPase domain by …
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Investigating the protein quality control pathways that prevent protein aggregation in the social amoeba Dictyostelium discoideum
… hallmark of many neurodegenerative diseases is protein aggregation. Protein aggregation occurs in part due to failure of protein quality control pathways. A better understanding of these pathways could allow us to improve therapies to help control protein aggregation. Here, I investigated …
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Degradation of the E. coli small heat-shock proteins by the AAA+ protease lon : significance to protein quality-control
… and elimination of damaged and aggregated proteins requires the concerted effort of several branches of the protein quality-control network. This network includes refolding chaperones, disaggregases, holdases and proteases. Many years of investigation have led to a partial understanding of …
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THE INTERPLAY BETWEEN THE PROTEIN QUALITY CONTROL SYSTEM AND EXTRACELLULAR VESICLES IN THE DISPOSAL OF DISEASE-ASSOCIATED PROTEINS AND MIRNAS IN ALS AND FTD MODELS
… the insoluble forms of the TAR DNA-binding protein of 43 KDa (TDP-43) and its C-terminal fragments (CTFs) of 35 (TDP-35) and 25 KDa (TDP-25). The accumulation of TDP-43 into insoluble cytoplasmic aggregates is toxic for cells that prevent their formation and/or promote their degradation …
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Aneuploidy reveals insights into control of protein complex stoichiometry
… physiology and places intense demand on the protein quality control system of the cell to effectively fold and/or degrade proteins. Aneuploidy further represents an ideal model for studying how cells cope with imbalances in their proteome as it allows for interrogation of the fate of hundreds …
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Regulation of Protein Degradation by ADP-Ribosylation
<p>Protein quality control is essential for cellular homeostasis. The Ubiquitin-Proteasome System (UPS) is responsible for the regulated breakdown of intracellular proteins. All proteins are not degraded at the same rate in the cell. For instance, global protein turnover rates in mammals change …
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Neuronal Protection by a Novel C-terminal Hsp90 Modulator
… by accumulation of misfolded and aggregated proteins, indicating that the protein quality control machinery is compromised. Enhancing the activity of molecular chaperones such as the `heat shock' proteins (Hsp's) that re-fold or signal degradation of damaged proteins may help remove protein …
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The role of Hsp60 in Amyloid beta toxicity: Relevance to Alzheimer’s disease
… Ab, a cleavage product of the amyloid precursor protein (APP). Furthermore, compelling evidence suggests that an age-related failure of the protein quality control machinery could also contribute to the onset of AD, thus supporting the hypothesis that the main risk factor of late-onset AD is …
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The role of Hsp60 in Amyloid beta toxicity: Relevance to Alzheimer’s disease
… Ab, a cleavage product of the amyloid precursor protein (APP). Furthermore, compelling evidence suggests that an age-related failure of the protein quality control machinery could also contribute to the onset of AD, thus supporting the hypothesis that the main risk factor of late-onset AD is …
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Hrd1 Partners In Endoplasmic Reticulum-Associated Degradation
<p>Protein Quality Control (PQC) comprises cellular pathways that regulate the turnover of short-lived, misfolded proteins. A main component of PQC is Endoplasmic Reticulum (ER)-Associated Degradation (ERAD), which controls the degradation of proteins synthesized in the ER. Aberrations in ERAD have …
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Role of the MecA adaptor protein in regulation of the AAA + chaperone ClpC of Bacillus subtilis
ClpC, a member of the AAA+ protein superfamily from Bacillus subtilis, is forming with ClpP a proteolytic system, that is part of the protein quality control system and involved in general proteolysis of misfolded and aggregated proteins. In addition ClpCP together with the adaptor MecA is …
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The Role of ATF5 in Mitochondrial Maintenance, Biogenesis and UPRmt Signaling Following Acute Exercise in Skeletal Muscle
Maintenance of the mitochondrial protein folding environment is essential for organellar and cellular homeostasis. The Mitochondrial Unfolded Protein Response (UPRmt) is a protein quality control mechanism that relieves intraorganellar proteotoxic stress in a manner dependent on activating …
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Nucleotide binding and conformational switching in the hexameric ring of a AAA+ machine
ATP-powered proteases enforce protein quality-control and regulation in all domains of life. ClpX, a AAA+ ring homohexamer, uses the energy of ATP binding and hydrolysis to power conformational changes that unfold and translocate target proteins into the ClpP peptidase for degradation. X-ray …
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Translational tuning pre-emptively modulates protein folding and secretory pathway defects
… against accumulation of aberrant membrane proteins caused by misfolding or secretory pathway disruption. Yor1, the yeast homolog of mammalian CFTR, encodes an ABC transporter that acts as a drug pump to extrude the mitochondrial toxin, oligomycin. A genome wide screen was previously …
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