Search Results - "HARDING, Heather P"

Selective Inhibition of a Regulatory Subunit of Protein Phosphatase 1 Restores Proteostasis by Tsaytler, Pavel, Harding, Heather P., Ron, David, Bertolotti, Anne

“…Many biological processes are regulated through the selective dephosphorylation of proteins. Protein serine-threonine phosphatases are assembled from catalytic…”
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Mutations in a translation initiation factor identify the target of a memory-enhancing compound by Sekine, Yusuke, Zyryanova, Alisa, Crespillo-Casado, Ana, Fischer, Peter M., Harding, Heather P., Ron, David

“…The integrated stress response (ISR) modulates messenger RNA translation to regulate the mammalian unfolded protein response (UPR), immunity, and memory…”
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A J-Protein Co-chaperone Recruits BiP to Monomerize IRE1 and Repress the Unfolded Protein Response by Amin-Wetzel, Niko, Saunders, Reuben A., Kamphuis, Maarten J., Rato, Claudia, Preissler, Steffen, Harding, Heather P., Ron, David

“…When unfolded proteins accumulate in the endoplasmic reticulum (ER), the unfolded protein response (UPR) increases ER-protein-folding capacity to restore…”
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Higher-order phosphatase–substrate contacts terminate the integrated stress response by Yan, Yahui, Harding, Heather P., Ron, David

“…Many regulatory PPP1R subunits join few catalytic PP1c subunits to mediate phosphoserine and phosphothreonine dephosphorylation in metazoans. Regulatory…”
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Binding of ISRIB reveals a regulatory site in the nucleotide exchange factor eIF2B by Zyryanova, Alisa F, Weis, Félix, Faille, Alexandre, Alard, Akeel Abo, Crespillo-Casado, Ana, Sekine, Yusuke, Harding, Heather P, Allen, Felicity, Parts, Leopold, Fromont, Christophe, Fischer, Peter M, Warren, Alan J, Ron, David

“…The integrated stress response (ISR) is a conserved translational and transcriptional program affecting metabolism, memory, and immunity. The ISR is mediated…”
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The ribosomal P-stalk couples amino acid starvation to GCN2 activation in mammalian cells by Harding, Heather P, Ordonez, Adriana, Allen, Felicity, Parts, Leopold, Inglis, Alison J, Williams, Roger L, Ron, David

“…The eukaryotic translation initiation factor 2α (eIF2α) kinase GCN2 is activated by amino acid starvation to elicit a rectifying physiological program known as…”
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molecular basis for selective inhibition of unconventional mRNA splicing by an IRE1-binding small molecule by Cross, Benedict C. S, Bond, Peter J, Sadowski, Pawel G, Jha, Babal Kant, Zak, Jaroslav, Goodman, Jonathan M, Silverman, Robert H, Neubert, Thomas A, Baxendale, Ian R, Ron, David, Harding, Heather P

“…IRE1 couples endoplasmic reticulum unfolded protein load to RNA cleavage events that culminate in the sequence-specific splicing of the Xbp1 mRNA and in the…”
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Ppp1r15 gene knockout reveals an essential role for translation initiation factor 2 alpha (eIF2α) dephosphorylation in mammalian development by Harding, Heather P, Zhang, Yuhong, Scheuner, Donalyn, Chen, Jane-Jane, Kaufman, Randal J, Ron, David

“…Diverse cellular stress responses are linked to phosphorylation of serine 51 on the alpha subunit of translation initiation factor 2. The resultant attenuation…”
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Paradoxical Sensitivity to an Integrated Stress Response Blocking Mutation in Vanishing White Matter Cells by Sekine, Yusuke, Zyryanova, Alisa, Crespillo-Casado, Ana, Amin-Wetzel, Niko, Harding, Heather P, Ron, David

“…The eukaryotic translation initiation factor eIF2B promotes mRNA translation as a guanine nucleotide exchange factor (GEF) for translation initiation factor 2…”
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Cargo receptor-assisted endoplasmic reticulum export of pathogenic α1-antitrypsin polymers by Ordóñez, Adriana, Harding, Heather P., Marciniak, Stefan J., Ron, David

“…Circulating polymers of α1-antitrypsin (α1AT) are neutrophil chemo-attractants and contribute to inflammation, yet cellular factors affecting their secretion…”
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Translation Reinitiation at Alternative Open Reading Frames Regulates Gene Expression in an Integrated Stress Response by Lu, Phoebe D., Harding, Heather P., Ron, David

“…Stress-induced eukaryotic translation initiation factor 2 (eIF2) α phosphorylation paradoxically increases translation of the metazoan activating transcription…”
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A genome-wide CRISPR/Cas9 screen identifies calreticulin as a selective repressor of ATF6α by Tung, Joanne, Huang, Lei, George, Ginto, Harding, Heather P, Ron, David, Ordonez, Adriana

“…Activating transcription factor 6 (ATF6) is one of three endoplasmic reticulum (ER) transmembrane stress sensors that mediate the unfolded protein response…”
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CHOP induces death by promoting protein synthesis and oxidation in the stressed endoplasmic reticulum by Marciniak, Stefan J, Yun, Chi Y, Oyadomari, Seiichi, Novoa, Isabel, Zhang, Yuhong, Jungreis, Rivka, Nagata, Kazuhiro, Harding, Heather P, Ron, David

“…Unfolded and malfolded client proteins impose a stress on the endoplasmic reticulum (ER), which contributes to cell death in pathophysiological conditions. The…”
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Role for the obesity-related FTO gene in the cellular sensing of amino acids by Gulati, Pawan, Cheung, Man Ka, Antrobus, Robin, Church, Chris D., Harding, Heather P., Tung, Yi-Chun Loraine, Rimmington, Debra, Ma, Marcella, Ron, David, Lehner, Paul J., Ashcroft, Frances M., Cox, Roger D., Coll, Anthony P., O'Rahilly, Stephen, Yeo, Giles S. H.

“…SNPs in the first intron of FTO (fat mass and obesity associated) are strongly associated with human obesity. While it is not yet formally established that…”
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Oligodendrocyte-specific activation of PERK signaling protects mice against experimental autoimmune encephalomyelitis by Lin, Wensheng, Lin, Yifeng, Li, Jin, Fenstermaker, Ali G, Way, Sharon W, Clayton, Benjamin, Jamison, Stephanie, Harding, Heather P, Ron, David, Popko, Brian

“…There is compelling evidence that oligodendrocyte apoptosis, in response to CNS inflammation, contributes significantly to the development of the demyelinating…”
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Flavonol Activation Defines an Unanticipated Ligand-Binding Site in the Kinase-RNase Domain of IRE1 by Wiseman, R. Luke, Zhang, Yuhong, Lee, Kenneth P.K., Harding, Heather P., Haynes, Cole M., Price, Joshua, Sicheri, Frank, Ron, David

“…Signaling in the most conserved branch of the endoplasmic reticulum (ER) unfolded protein response (UPR) is initiated by sequence-specific cleavage of the…”
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Infancy‐onset diabetes caused by de‐regulated AMPylation of the human endoplasmic reticulum chaperone BiP by Perera, Luke A, Hattersley, Andrew T, Harding, Heather P, Wakeling, Matthew N, Flanagan, Sarah E, Mohsina, Ibrahim, Raza, Jamal, Gardham, Alice, Ron, David, De Franco, Elisa

“…Dysfunction of the endoplasmic reticulum (ER) in insulin‐producing beta cells results in cell loss and diabetes mellitus. Here we report on five individuals…”
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Ubiquitin-like protein 5 positively regulates chaperone gene expression in the mitochondrial unfolded protein response by Benedetti, Cristina, Haynes, Cole M, Yang, Yun, Harding, Heather P, Ron, David

“…Perturbation of the protein-folding environment in the mitochondrial matrix selectively upregulates the expression of nuclear genes encoding mitochondrial…”
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Targeting the unfolded protein response in disease by Hetz, Claudio, Chevet, Eric, Harding, Heather P.

“…Key Points The endoplasmic reticulum (ER) is the main organelle involved in protein folding, and different perturbations to its function lead to the…”
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Lifetime imaging of a fluorescent protein sensor reveals surprising stability of ER thiol redox by Avezov, Edward, Cross, Benedict C S, Kaminski Schierle, Gabriele S, Winters, Mikael, Harding, Heather P, Melo, Eduardo Pinho, Kaminski, Clemens F, Ron, David

“…Interfering with disulfide bond formation impedes protein folding and promotes endoplasmic reticulum (ER) stress. Due to limitations in measurement techniques,…”
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