Suchergebnisse - "Lin, Jonathan H."

ER stress and unfolded protein response in ocular health and disease von Kroeger, Heike, Chiang, Wei‐Chieh, Felden, Julia, Nguyen, Amanda, Lin, Jonathan H.

“… The human eye is the organ that is able to react to light in order to provide sharp three‐dimensional and colored images. Unfortunately, the health of the eye …”
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Multiple Mechanisms of Unfolded Protein Response-Induced Cell Death von Hiramatsu, Nobuhiko, Chiang, Wei-Chieh, Kurt, Timothy D, Sigurdson, Christina J, Lin, Jonathan H

“… Eukaryotic cells fold and assemble membrane and secreted proteins in the endoplasmic reticulum (ER), before delivery to other cellular compartments or the …”
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Regulated Ire1-dependent decay of messenger RNAs in mammalian cells von Hollien, Julie, Lin, Jonathan H, Li, Han, Stevens, Nicole, Walter, Peter, Weissman, Jonathan S

“… Maintenance of endoplasmic reticulum (ER) function is achieved in part through Ire1 (inositol-requiring enzyme 1), a transmembrane protein activated by protein …”
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Divergent Effects of PERK and IRE1 Signaling on Cell Viability von Lin, Jonathan H., Li, Han, Zhang, Yuhong, Ron, David, Walter, Peter

“… Protein misfolding in the endoplasmic reticulum (ER) activates a set of intracellular signaling pathways, collectively termed the Unfolded Protein Response …”
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IRE1 signaling affects cell fate during the unfolded protein response von Lin, Jonathan H, Li, Han, Yasumura, Douglas, Cohen, Hannah R, Zhang, Chao, Panning, Barbara, Shokat, Kevan M, Lavail, Matthew M, Walter, Peter

“… Endoplasmic reticulum (ER) stress activates a set of signaling pathways, collectively termed the unfolded protein response (UPR). The three UPR branches (IRE1, …”
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ATF6 is required for efficient rhodopsin clearance and retinal homeostasis in the P23H rho retinitis pigmentosa mouse model von Lee, Eun-Jin, Chan, Priscilla, Chea, Leon, Kim, Kyle, Kaufman, Randal J., Lin, Jonathan H.

“… Retinitis Pigmentosa (RP) is a blinding disease that arises from loss of rods and subsequently cones. The P23H rhodopsin knock-in (P23H-KI) mouse develops …”
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Intercellular transmission of the unfolded protein response promotes survival and drug resistance in cancer cells von Rodvold, Jeffrey J, Chiu, Kevin T, Hiramatsu, Nobuhiko, Nussbacher, Julia K, Galimberti, Valentina, Mahadevan, Navin R, Willert, Karl, Lin, Jonathan H, Zanetti, Maurizio

“… Increased protein translation in cells and various factors in the tumor microenvironment can induce endoplasmic reticulum (ER) stress, which initiates the …”
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Rates and timing of subsequent amputation after initial minor amputation von Lin, Jonathan H, Jeon, Sun Young, Romano, Patrick S, Humphries, Misty D

“… Studies evaluating major amputation after initial minor amputation are few with rates of subsequent major amputation ranging from 14% to 35% with limited …”
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Endoplasmic reticulum stress in disease pathogenesis von Lin, Jonathan H, Walter, Peter, Yen, T S Benedict

“… The endoplasmic reticulum (ER) is the site of synthesis and folding of membrane and secretory proteins, which, collectively, represent a large fraction of the …”
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ATF6 is essential for human cone photoreceptor development von Kroeger, Heike, Grandjean, Julia M D, Chiang, Wei-Chieh Jerry, Bindels, Daphne D, Mastey, Rebecca, Okalova, Jennifer, Nguyen, Amanda, Powers, Evan T, Kelly, Jeffery W, Grimsey, Neil J, Michaelides, Michel, Carroll, Joseph, Wiseman, R Luke, Lin, Jonathan H

“… Endoplasmic reticulum (ER) stress and Unfolded Protein Response (UPR) signaling promote the pathology of many human diseases. Loss-of-function variants of the …”
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Mutations in unfolded protein response regulator ATF6 cause hearing and vision loss syndrome von Lee, Eun-Jin, Kim, Kyle, Diaz-Aguilar, Monica Sophia, Min, Hyejung, Chavez, Eduardo, Steinbergs, Korina J., Safarta, Lance A., Zhang, Guirong, Ryan, Allen F., Lin, Jonathan H.

“… Activating transcription factor 6 (ATF6) is a key regulator of the unfolded protein response (UPR) and is important for ER function and protein homeostasis in …”
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Robust Endoplasmic Reticulum-Associated Degradation of Rhodopsin Precedes Retinal Degeneration von Chiang, Wei-Chieh, Kroeger, Heike, Sakami, Sanae, Messah, Carissa, Yasumura, Douglas, Matthes, Michael T., Coppinger, Judith A., Palczewski, Krzysztof, LaVail, Matthew M., Lin, Jonathan H.

“… Rhodopsin is a G protein-coupled receptor essential for vision and rod photoreceptor viability. Disease-associated rhodopsin mutations, such as P23H rhodopsin, …”
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Targeting ATF6 reduces pathological neovascularization and improves visual outcomes in retinal disease models von Bradley, Allyssa, Park, Soyoung, Park, Soyeon, Kim, Kyle, Galdamez, Angela, Min, Hyejung, Diaz-Aguilar, Monica Sophia, Hartnett, M. Elizabeth, Lee, Eun-Jin, Lin, Jonathan H.

“… Pathological retinal neovascularization is a cause of vision loss in diseases including retinopathy of prematurity (ROP), wet age-related macular degeneration …”
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Characterization of Retinal Structure in ATF6 -Associated Achromatopsia von Mastey, Rebecca R., Georgiou, Michalis, Langlo, Christopher S., Kalitzeos, Angelos, Patterson, Emily J., Kane, Thomas, Singh, Navjit, Vincent, Ajoy, Moore, Anthony T., Tsang, Stephen H., Lin, Jonathan H., Young, Marielle P., Hartnett, M. Elizabeth, Héon, Elise, Kohl, Susanne, Michaelides, Michel, Carroll, Joseph

“… Mutations in six genes have been associated with achromatopsia (ACHM): CNGA3, CNGB3, PDE6H, PDE6C, GNAT2, and ATF6. ATF6 is the most recent gene to be …”
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Restoration of visual function in P23H rhodopsin transgenic rats by gene delivery of BiP/Grp78 von Gorbatyuk, Marina S, Knox, Tessa, LaVail, Matthew M, Gorbatyuk, Oleg S, Noorwez, Syed M, Hauswirth, William W, Lin, Jonathan H, Muzyczka, Nicholas, Lewin, Alfred S

“… The P23H mutation within the rhodopsin gene (RHO) causes rhodopsin misfolding, endoplasmic reticulum (ER) stress, and activates the unfolded protein response …”
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Uveal melanoma following Bruton's tyrosine inhibitor use for the treatment of hematologic malignancies von Murty, Tara, Lin, Jonathan H., Mruthyunjaya, Prithvi

“… To report two new cases and summarize one case of uveal melanoma following treatment of hematologic malignancies with Bruton's tyrosine kinase inhibitors. A …”
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IRE1 directs proteasomal and lysosomal degradation of misfolded rhodopsin von Chiang, Wei-Chieh, Messah, Carissa, Lin, Jonathan H

“… Endoplasmic reticulum (ER) is responsible for folding of secreted and membrane proteins in eukaryotic cells. Disruption of ER protein folding leads to ER …”
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Selective activation of ATF6 and PERK endoplasmic reticulum stress signaling pathways prevent mutant rhodopsin accumulation von Chiang, Wei-Chieh, Hiramatsu, Nobuhiko, Messah, Carissa, Kroeger, Heike, Lin, Jonathan H

“… Many rhodopsin mutations that cause retinitis pigmentosa produce misfolded rhodopsin proteins that are retained within the endoplasmic reticulum (ER) and cause …”
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Network biology analysis of P23H rhodopsin interactome identifies protein and mRNA quality control mechanisms von Kim, Kyle, Safarta, Lance A., Chiang, Wei-Chieh J., Coppinger, Judith A., Lee, Eun-Jin, Lin, Jonathan H.

“… Rhodopsin is essential for phototransduction, and many rhodopsin mutations cause heritable retinal degenerations. The P23H rhodopsin variant generates a …”
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Prion Seeds Distribute throughout the Eyes of Sporadic Creutzfeldt-Jakob Disease Patients von Orrù, Christina D., Soldau, Katrin, Cordano, Christian, Llibre-Guerra, Jorge, Green, Ari J., Sanchez, Henry, Groveman, Bradley R., Edland, Steven D., Safar, Jiri G., Lin, Jonathan H., Caughey, Byron, Geschwind, Michael D., Sigurdson, Christina J.

“… Cases of iatrogenic prion disease have been reported from corneal transplants, yet the distribution and levels of prions throughout the eye remain unknown …”
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