Search Results - "Lin, Jonathan H."
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Multiple Mechanisms of Unfolded Protein Response–Induced Cell Death
Published in The American journal of pathology (01-07-2015)“…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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ER stress and unfolded protein response in ocular health and disease
Published in The FEBS journal (01-01-2019)“…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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Regulated Ire1-dependent decay of messenger RNAs in mammalian cells
Published in The Journal of cell biology (10-08-2009)“…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
Published in PloS one (12-01-2009)“…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
Published in Science (American Association for the Advancement of Science) (09-11-2007)“…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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Intercellular transmission of the unfolded protein response promotes survival and drug resistance in cancer cells
Published in Science signaling (06-06-2017)“…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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Endoplasmic reticulum stress in disease pathogenesis
Published in Annual review of pathology (01-01-2008)“…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 required for efficient rhodopsin clearance and retinal homeostasis in the P23H rho retinitis pigmentosa mouse model
Published in Scientific reports (11-08-2021)“…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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Rates and timing of subsequent amputation after initial minor amputation
Published in Journal of vascular surgery (01-07-2020)“…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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Robust Endoplasmic Reticulum-Associated Degradation of Rhodopsin Precedes Retinal Degeneration
Published in Molecular neurobiology (01-08-2015)“…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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Restoration of visual function in P23H rhodopsin transgenic rats by gene delivery of BiP/Grp78
Published in Proceedings of the National Academy of Sciences - PNAS (30-03-2010)“…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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Characterization of Retinal Structure in ATF6-Associated Achromatopsia
Published in Investigative ophthalmology & visual science (01-06-2019)“…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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IRE1 directs proteasomal and lysosomal degradation of misfolded rhodopsin
Published in Molecular biology of the cell (01-03-2012)“…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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Network biology analysis of P23H rhodopsin interactome identifies protein and mRNA quality control mechanisms
Published in Scientific reports (18-10-2022)“…Rhodopsin is essential for phototransduction, and many rhodopsin mutations cause heritable retinal degenerations. The P23H rhodopsin variant generates a…”
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Selective activation of ATF6 and PERK endoplasmic reticulum stress signaling pathways prevent mutant rhodopsin accumulation
Published in Investigative ophthalmology & visual science (01-10-2012)“…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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Prion Seeds Distribute throughout the Eyes of Sporadic Creutzfeldt-Jakob Disease Patients
Published in mBio (20-11-2018)“…Sporadic Creutzfeldt-Jakob disease (sCJD) is the most common prion disease in humans and has been iatrogenically transmitted through corneal graft…”
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Interpreting Discordant Monosomy 3 FISH and Chromosomal Microarray Analysis Results in Uveal Melanoma
Published in Diagnostics (Basel) (01-03-2023)“…Uveal melanoma is the most common primary ocular tumor in adults and causes morbidity through lymphovascular metastasis. The presence of monosomy 3 in uveal…”
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IRE1α and IGF signaling predict resistance to an endoplasmic reticulum stress-inducing drug in glioblastoma cells
Published in Scientific reports (20-05-2020)“…To date current therapies of glioblastoma multiforme (GBM) are largely ineffective. The induction of apoptosis by an unresolvable unfolded protein response…”
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Conjunctival blue nevus in a child – Case report and review of literature
Published in American journal of ophthalmology case reports (01-12-2024)“…To report a rare case of a conjunctival blue nevus in a child. A 10-year-old girl underwent an excisional biopsy for an atypical growing melanocytic…”
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ER stress in retinal degeneration in S334ter Rho rats
Published in PloS one (14-03-2012)“…The S334ter rhodopsin (Rho) rat (line 4) bears the rhodopsin gene with an early termination codon at residue 334 that is a model for several such mutations…”
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