Results 21 to 30 of about 28,738 (248)

Achromatopsia mutations target sequential steps of ATF6 activation [PDF]

Proceedings of the National Academy of Sciences, 2016
Significance The unfolded protein response regulator activating transcription factor 6 ( ATF6 ) was recently identified as a novel genetic cause of the cone photoreceptor disease achromatopsia. ATF6 upregulates genes that help cells cope with endoplasmic reticulum stress.
Wei‐Chieh Chiang, Priscilla Chan, Bernd Wissinger, Ajoy Vincent, Anna Skorczyk‐Werner, M Krawczyński, Randal J. Kaufman, Stephen H. Tsang, Elise Héon, Susanne Kohl, Jonathan H. Lin   +10 more
openalex   +3 more sources

ATF6 modulates SREBP2-mediated lipogenesis [PDF]

The EMBO Journal, 2004
Activating transcription factor 6 (ATF6) and sterol regulatory element-binding proteins (SREBPs) are activated by proteolytic cleavage. The ensuing nuclear translocation of their N-termini (i.e., ATF6(N) and SREBP(N)) activates the respective target genes involved in unfolded protein response and lipogenesis.
Zeng, L, Lu, M, Mori, K, Luo, S, Lee, A S, Zhu, Y, Shyy, J Y-J   +6 more
openaire   +4 more sources

Designing Novel Therapies to Mend Broken Hearts: ATF6 and Cardiac Proteostasis

Cells, 2020
The heart exhibits incredible plasticity in response to both environmental and genetic alterations that affect workload. Over the course of development, or in response to physiological or pathological stimuli, the heart responds to fluctuations in ...
Erik A. Blackwood, Alina S. Bilal, Winston T. Stauffer, Adrian Arrieta, Christopher C. Glembotski   +4 more
doaj   +1 more source

BiP Binding Keeps ATF6 at Bay [PDF]

Developmental Cell, 2002
A study by, in this issue of Developmental Cell shows that transport to the Golgi complex and subsequent proteolytic activation of the stress-regulated transcription factor ATF6 is initiated by the dissociation of the ER chaperone BiP from ATF6. This demonstrates that BiP is a key element in sensing the folding capacity within the ER and provides ...
Sommer, Thomas, Jarosch, Ernst
openaire   +2 more sources

ATF6 is required for efficient rhodopsin clearance and retinal homeostasis in the P23H rho retinitis pigmentosa mouse model

Scientific Reports, 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 retinal degeneration that mirrors RP phenotype in patients carrying the orthologous variant ...
Eun-Jin Lee, Priscilla Chan, Leon Chea, Kyle Kim, Randal J. Kaufman, Jonathan H. Lin   +5 more
doaj   +1 more source

Establishment and validation of an endoplasmic reticulum stress reporter to monitor zebrafish ATF6 activity in development and disease

Disease Models & Mechanisms, 2020
Induction of endoplasmic reticulum (ER) stress is associated with diverse developmental and degenerative diseases. Modified ER homeostasis causes activation of conserved stress pathways at the ER called the unfolded protein response (UPR).
Eric M. Clark, Hannah J. T. Nonarath, Jonathan R. Bostrom, Brian A. Link   +3 more
doaj   +1 more source

TP53 R249S mutation in hepatic organoids captures the predisposing cancer risk

Hepatology, EarlyView., 2022
The systematic approach in elucidating the gain‐of‐function (GOF) roles of TP53 mutations in early liver carcinogenesis. Unique downstream targets of TP53 L3 mutations were identified from chormatin immunoprecipitation sequencing in HCC cell lines, followed by a series of validation assays to substantiate the exclusive transcriptional regulations ...
Yin Kau Lam, Jianqing Yu, Hao Huang, Xiaofan Ding, Alissa M. Wong, Howard H. Leung, Anthony W. Chan, Kelvin K. Ng, Mingjing Xu, Xin Wang, Nathalie Wong   +10 more
wiley   +1 more source

Pharmacological Activating Transcription Factor 6 Activation Is Beneficial for Liver Retrieval With ex vivo Normothermic Mechanical Perfusion From Cardiac Dead Donor Rats

Frontiers in Surgery, 2021
Background: Normothermic machine perfusion (NMP) could be beneficial for organ retrieval from donors after cardiac death (DCD). Activating transcription factor 6 (ATF6) was recently shown to mitigate liver ischemia/reperfusion injury and confer ...
Nuo Cheng, Ji-Hua Shi, Yang Jin, Yuan-Bin Shi, Xu-Dong Liu, Hua-Peng Zhang, Sheng-Li Cao, Han Yang, Wen-Zhi Guo, Shui-Jun Zhang   +9 more
doaj   +1 more source

Proteostasis and Beyond: ATF6 in Ischemic Disease [PDF]

Trends in Molecular Medicine, 2019
Endoplasmic reticulum (ER) stress is a pathological hallmark of numerous ischemic diseases, including stroke and myocardial infarction (MI). In these diseases, ER stress leads to activation of the unfolded protein response (UPR) and subsequent adaptation of cellular physiology in ways that dictate cellular fate following ischemia.
Christopher C. Glembotski, Jessica D. Rosarda, R. Luke Wiseman   +2 more
openaire   +2 more sources

Macular maldevelopment inATF6-mediated retinal dysfunction [PDF]

Ophthalmic Genetics, 2019
Background: Achromatopsia has been previously associated with mutations in the ATF6 gene. Rod-monochromatism, foveal hypoplasia, and disruption of the subfoveal photoreceptor layer are described as phenotypical features. We report detailed structural and electrophysiological assessment of two patients from two families, one manifesting severe macular ...
Markus Ritter, Gavin Arno, Rola Ba-Abbad, Graham E. Holder, Andrew R. Webster   +4 more
openaire   +3 more sources