Search Results - "Fregno, Ilaria"

Proteasomal and lysosomal clearance of faulty secretory proteins: ER-associated degradation (ERAD) and ER-to-lysosome-associated degradation (ERLAD) pathways by Fregno, Ilaria, Molinari, Maurizio

“…About 40% of the eukaryotic cell's proteins are inserted co- or post-translationally in the endoplasmic reticulum (ER), where they attain the native structure…”
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N‐glycan processing selects ERAD‐resistant misfolded proteins for ER‐to‐lysosome‐associated degradation by Fregno, Ilaria, Fasana, Elisa, Soldà, Tatiana, Galli, Carmela, Molinari, Maurizio

“…Efficient degradation of by‐products of protein biogenesis maintains cellular fitness. Strikingly, the major biosynthetic compartment in eukaryotic cells, the…”
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Endoplasmic reticulum turnover: ER-phagy and other flavors in selective and non-selective ER clearance [version 1; peer review: 2 approved] by Fregno, Ilaria, Molinari, Maurizio

“…The endoplasmic reticulum (ER) is a highly dynamic organelle in eukaryotic cells. It is deputed to lipid and protein biosynthesis, calcium storage, and the…”
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Translocon component Sec62 acts in endoplasmic reticulum turnover during stress recovery by Fumagalli, Fiorenza, Noack, Julia, Bergmann, Timothy J., Cebollero, Eduardo, Pisoni, Giorgia Brambilla, Fasana, Elisa, Fregno, Ilaria, Galli, Carmela, Loi, Marisa, Soldà, Tatiana, D’Antuono, Rocco, Raimondi, Andrea, Jung, Martin, Melnyk, Armin, Schorr, Stefan, Schreiber, Anne, Simonelli, Luca, Varani, Luca, Wilson-Zbinden, Caroline, Zerbe, Oliver, Hofmann, Kay, Peter, Matthias, Quadroni, Manfredo, Zimmermann, Richard, Molinari, Maurizio

“…The endoplasmic reticulum (ER) is a site of protein biogenesis in eukaryotic cells. Perturbing ER homeostasis activates stress programs collectively called the…”
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Validation of a highly sensitive HaloTag-based assay to evaluate the potency of a novel class of allosteric β-Galactosidase correctors by Rudinskiy, Mikhail, Pons-Vizcarra, Maria, Soldà, Tatiana, Fregno, Ilaria, Bergmann, Timothy Jan, Ruano, Ana, Delgado, Aida, Morales, Sara, Barril, Xavier, Bellotto, Manolo, Cubero, Elena, García-Collazo, Ana María, Pérez-Carmona, Natalia, Molinari, Maurizio

“…Site-directed Enzyme Enhancement Therapy (SEE-Tx®) technology is a disease-agnostic drug discovery tool that can be applied to any protein target of interest…”
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Erratum: Corrigendum: Translocon component Sec62 acts in endoplasmic reticulum turnover during stress recovery by Fumagalli, Fiorenza, Noack, Julia, Bergmann, Timothy J., Cebollero, Eduardo, Pisoni, Giorgia Brambilla, Fasana, Elisa, Fregno, Ilaria, Galli, Carmela, Loi, Marisa, Soldà, Tatiana, D'Antuono, Rocco, Raimondi, Andrea, Jung, Martin, Melnyk, Armin, Schorr, Stefan, Schreiber, Anne, Simonelli, Luca, Varani, Luca, Wilson-Zbinden, Caroline, Zerbe, Oliver, Hofmann, Kay, Peter, Matthias, Quadroni, Manfredo, Zimmermann, Richard, Molinari, Maurizio

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ER‐to‐lysosome‐associated degradation of proteasome‐resistant ATZ polymers occurs via receptor‐mediated vesicular transport by Fregno, Ilaria, Fasana, Elisa, Bergmann, Timothy J, Raimondi, Andrea, Loi, Marisa, Soldà, Tatiana, Galli, Carmela, D'Antuono, Rocco, Morone, Diego, Danieli, Alberto, Paganetti, Paolo, van Anken, Eelco, Molinari, Maurizio

“…Maintenance of cellular proteostasis relies on efficient clearance of defective gene products. For misfolded secretory proteins, this involves dislocation from…”
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ER-to-lysosome-associated degradation acts as failsafe mechanism upon ERAD dysfunction by Fasana, Elisa, Fregno, Ilaria, Galli, Carmela, Soldà, Tatiana, Molinari, Maurizio

“…The endoplasmic reticulum (ER) produces proteins destined to organelles of the endocytic and secretory pathways, the plasma membrane, and the extracellular…”
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A selective ER‐phagy exerts procollagen quality control via a Calnexin‐FAM134B complex by Forrester, Alison, De Leonibus, Chiara, Grumati, Paolo, Fasana, Elisa, Piemontese, Marilina, Staiano, Leopoldo, Fregno, Ilaria, Raimondi, Andrea, Marazza, Alessandro, Bruno, Gemma, Iavazzo, Maria, Intartaglia, Daniela, Seczynska, Marta, van Anken, Eelco, Conte, Ivan, De Matteis, Maria Antonietta, Dikic, Ivan, Molinari, Maurizio, Settembre, Carmine

“…Autophagy is a cytosolic quality control process that recognizes substrates through receptor‐mediated mechanisms. Procollagens, the most abundant gene products…”
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Chemical stresses fail to mimic the unfolded protein response resulting from luminal load with unfolded polypeptides by Bergmann, Timothy J., Fregno, Ilaria, Fumagalli, Fiorenza, Rinaldi, Andrea, Bertoni, Francesco, Boersema, Paul J., Picotti, Paola, Molinari, Maurizio

“…The stress sensors ATF6, IRE1, and PERK monitor deviations from homeostatic conditions in the endoplasmic reticulum (ER), a protein biogenesis compartment of…”
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Validation of a highly sensitive HaloTag-based assay to evaluate the potency of a novel class of allosteric [beta]-Galactosidase correctors by Rudinskiy, Mikhail, Pons-Vizcarra, Maria, Soldà, Tatiana, Fregno, Ilaria, Bergmann, Timothy Jan, Ruano, Ana, Delgado, Aida, Morales, Sara, Barril, Xavier, Bellotto, Manolo, Cubero, Elena, García-Collazo, Ana María, Pérez-Carmona, Natalia, Molinari, Maurizio

“…Site-directed Enzyme Enhancement Therapy (SEE-Tx®) technology is a disease-agnostic drug discovery tool that can be applied to any protein target of interest…”
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Schwann cells ER-associated degradation contributes to myelin maintenance in adult nerves and limits demyelination in CMT1B mice by Volpi, Vera G, Ferri, Cinzia, Fregno, Ilaria, Del Carro, Ubaldo, Bianchi, Francesca, Scapin, Cristina, Pettinato, Emanuela, Solda, Tatiana, Feltri, M Laura, Molinari, Maurizio, Wrabetz, Lawrence, D'Antonio, Maurizio

“…In the peripheral nervous system (PNS) myelinating Schwann cells synthesize large amounts of myelin protein zero (P0) glycoprotein, an abundant component of…”
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A selective ER ‐phagy exerts procollagen quality control via a Calnexin‐ FAM 134B complex by Forrester, Alison, De Leonibus, Chiara, Grumati, Paolo, Fasana, Elisa, Piemontese, Marilina, Staiano, Leopoldo, Fregno, Ilaria, Raimondi, Andrea, Marazza, Alessandro, Bruno, Gemma, Iavazzo, Maria, Intartaglia, Daniela, Seczynska, Marta, van Anken, Eelco, Conte, Ivan, De Matteis, Maria Antonietta, Dikic, Ivan, Molinari, Maurizio, Settembre, Carmine

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Correction: Corrigendum: Translocon component Sec62 acts in endoplasmic reticulum turnover during stress recovery by Fumagalli, Fiorenza, Noack, Julia, Bergmann, Timothy J., Cebollero, Eduardo, Pisoni, Giorgia Brambilla, Fasana, Elisa, Fregno, Ilaria, Galli, Carmela, Loi, Marisa, Soldà, Tatiana, D'Antuono, Rocco, Raimondi, Andrea, Jung, Martin, Melnyk, Armin, Schorr, Stefan, Schreiber, Anne, Simonelli, Luca, Varani, Luca, Wilson-Zbinden, Caroline, Zerbe, Oliver, Hofmann, Kay, Peter, Matthias, Quadroni, Manfredo, Zimmermann, Richard, Molinari, Maurizio

“…Nature Cell Biology 18, 1173–1184 (2016); published online 17 October 2016; corrected after print 18 November 2016. In the version of this Article originally…”
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Corrigendum: Translocon component Sec62 acts in endoplasmic reticulum turnover during stress recovery by Fumagalli, Fiorenza, Noack, Julia, Bergmann, Timothy J, Cebollero, Eduardo, Pisoni, Giorgia Brambilla, Fasana, Elisa, Fregno, Ilaria, Galli, Carmela, Loi, Marisa, Soldà, Tatiana, D'Antuono, Rocco, Raimondi, Andrea, Jung, Martin, Melnyk, Armin, Schorr, Stefan, Schreiber, Anne, Simonelli, Luca, Varani, Luca, Wilson-Zbinden, Caroline, Zerbe, Oliver, Hofmann, Kay, Peter, Matthias, Quadroni, Manfredo, Zimmermann, Richard, Molinari, Maurizio

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