Search Results - "Wei-Chieh Chiang"
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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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2
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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3
The unfolded protein response regulator ATF6 promotes mesodermal differentiation
Published in Science signaling (13-02-2018)“…encodes a transcription factor that is anchored in the endoplasmic reticulum (ER) and activated during the unfolded protein response (UPR) to protect cells…”
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4
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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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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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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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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A 45 nm Stacked CMOS Image Sensor Process Technology for Submicron Pixel
Published in Sensors (Basel, Switzerland) (05-12-2017)“…A submicron pixel's light and dark performance were studied by experiment and simulation. An advanced node technology incorporated with a stacked CMOS image…”
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The effects of IRE1, ATF6, and PERK signaling on adRP-linked rhodopsins
Published in Advances in experimental medicine and biology (2014)“…Many mutations in rhodopsin gene linked to retinitis pigmentosa (RP) cause rhodopsin misfolding. Rod photoreceptor cells expressing misfolded rhodopsin…”
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10
De Novo Prion Aggregates Trigger Autophagy in Skeletal Muscle
Published in Journal of Virology (01-02-2014)“…Classifications Services JVI Citing Articles Google Scholar PubMed Related Content Social Bookmarking CiteULike Delicious Digg Facebook Google+ Mendeley Reddit…”
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11
Transmembrane domain interactions affect the stability of the extracellular domain of the human NTPDase 2
Published in Archives of biochemistry and biophysics (15-04-2008)“…Human NTPDase2 and chicken NTPDase8 are cell surface nucleotidases that contain two transmembrane domains (TMD) and five apyrase conserved regions (ACRs). ACR1…”
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12
Multiexon deletion alleles of ATF6 linked to achromatopsia
Published in JCI insight (09-04-2020)“…Achromatopsia (ACHM) is an autosomal recessive disease that results in severe visual loss. Symptoms of ACHM include impaired visual acuity, nystagmus, and…”
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13
Endoplasmic reticulum stress in human photoreceptor diseases
Published in Brain research (01-10-2016)“…Abstract Photoreceptors are specialized sensory neurons essential for light detection in the human eye. Photoreceptor cell dysfunction and death cause vision…”
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14
Enzymatic and transcriptional regulation of human ecto-ATPase/E-NTPDase 2
Published in Archives of biochemistry and biophysics (15-10-2003)“…We have characterized the regulation of expressed human ecto-ATPase (E-NTPDase 2), a cell surface integral membrane glycoprotein. Ecto-ATPase activity is…”
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15
Male germ cell-associated kinase is required for axoneme formation during ciliogenesis in zebrafish photoreceptors
Published in Disease models & mechanisms (01-07-2024)“…Vertebrate photoreceptors are highly specialized retinal neurons that have cilium-derived membrane organelles called outer segments, which function as…”
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ATF6 is essential for human cone photoreceptor development
Published in Proceedings of the National Academy of Sciences - PNAS (28-09-2021)“…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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Achromatopsia mutations target sequential steps of ATF6 activation
Published in Proceedings of the National Academy of Sciences - PNAS (10-01-2017)“…Achromatopsia is an autosomal recessive disorder characterized by cone photoreceptor dysfunction. We recently identified activating transcription factor 6…”
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Pathomechanisms of ATF6-Associated Cone Photoreceptor Diseases
Published in Advances in experimental medicine and biology (2019)“…Activating transcription factor 6 (ATF6) is a key regulator of the unfolded protein response (UPR). In response to endoplasmic reticulum (ER) stress, ATF6 is…”
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Autosomal recessive cone-rod dystrophy can be caused by mutations in the ATF6 gene
Published in European journal of human genetics : EJHG (01-11-2017)“…Inherited retinal dystrophies (IRDs) are clinically and genetically highly heterogeneous, making clinical diagnosis difficult. The advances in high-throughput…”
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Induction of endoplasmic reticulum stress genes, BiP and chop, in genetic and environmental models of retinal degeneration
Published in Investigative ophthalmology & visual science (09-11-2012)“…Endoplasmic reticulum (ER) stress has been observed in animal models of retinitis pigmentosa expressing P23H rhodopsin. We compared levels of tightly induced…”
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