Results 111 to 120 of about 9,109,989 (294)

Unraveling 4‐Phenylbutyrate's Therapeutic Role in SLC6A1 Disorders: Pharmacochaperoning Over HDAC Inhibition

Annals of Clinical and Translational Neurology, EarlyView.
ABSTRACT Objective Variants in SLC6A1, encoding the GABA transporter 1 (GAT‐1), cause epilepsy, autism spectrum disorder, and developmental delay via loss of GABA uptake, impaired trafficking, and ER retention. We previously found that 4‐Phenylbutyrate (PBA), an FDA‐approved drug, restores GABA uptake and reduces seizures in SLC6A1‐related disorders ...
Melissa B. DeLeeuw, Karishma Randhave, Ekta Anand, Ziang (Debbie) Song, Wangzhen Shen, Jing‐Qiong Kang   +5 more
wiley   +1 more source

Multiple Genes Core to ERAD, Macroautophagy and Lysosomal Degradation Pathways Participate in the Proteostasis Response in α1-Antitrypsin DeficiencySummary

Cellular and Molecular Gastroenterology and Hepatology
Background & Aims: In the classic form of α1-antitrypsin deficiency (ATD), the misfolded α1-antitrypsin Z (ATZ) variant accumulates in the endoplasmic reticulum (ER) of liver cells.
Jie Li, Francesca Moretti, Tunda Hidvegi, Sanja Sviben, James A.J. Fitzpatrick, Hemalatha Sundaramoorthi, Stephen C. Pak, Gary A. Silverman, Britta Knapp, Ireos Filipuzzi, John Alford, John Reece-Hoyes, Florian Nigsch, Leon O. Murphy, Beat Nyfeler, David H. Perlmutter   +15 more
doaj   +1 more source

ER-associated degradation regulates Alzheimer’s amyloid pathology and memory function by modulating γ-secretase activity

Nature Communications, 2017
Endoplasmic-reticulum associated degradation (ERAD) regulates protein homeostasis. Here the authors identify an ERAD component membralin, and show that it interacts with a member of the γ-secretase complex to regulate β-amyloid (Aβ) pathology and memory ...
Bing Zhu, LuLin Jiang, Timothy Huang, Yingjun Zhao, Tongfei Liu, Yongwang Zhong, Xiaoguang Li, Alexandre Campos, Kenneth Pomeroy, Eliezer Masliah, Dongxian Zhang, Huaxi Xu   +11 more
doaj   +1 more source

Patient‐Derived 3D Heart‐On‐a‐Chip Model of Dilated Cardiomyopathy With Embedded Bead‐Based Mapping of Tissue Contractility

Advanced Healthcare Materials, EarlyView.
A heart‐on‐a‐chip model of dilated cardiomyopathy is developed from patient‐derived induced pluripotent stem cells. The model recapitulates key disease phenotypes and enables functional assessment through integrated bead‐based tracking and pillar deflection measurements.
Ali Mousavi, Ludovic Mouttet, Shihao Cui, Yasaman Hekmatnia, Mehran Mottahedi, Ida Derish, Naimeh Rafatian, Mark Aurousseau, Gregor Andelfinger, Renzo Cecere, Houman Savoji   +10 more
wiley   +1 more source

Regulation and function of endoplasmic reticulum autophagy in neurodegenerative diseases

Neural Regeneration Research
The endoplasmic reticulum, a key cellular organelle, regulates a wide variety of cellular activities. Endoplasmic reticulum autophagy, one of the quality control systems of the endoplasmic reticulum, plays a pivotal role in maintaining endoplasmic ...
Xiu-Yun Zhao, De-En Xu, Ming-Lei Wu, Ji-Chuan Liu, Zi-Ling Shi, Quan-Hong Ma   +5 more
doaj   +1 more source

SYVN1, an ERAD E3 Ubiquitin Ligase, Is Involved in GABAAα1 Degradation Associated with Methamphetamine-Induced Conditioned Place Preference

Frontiers in Molecular Neuroscience, 2017
Abuse of methamphetamine (METH), a powerful addictive amphetamine-type stimulants (ATS), is becoming a global public health problem. The gamma-aminobutyric acid (GABA)ergic system plays a critical role in METH use disorders. By using rat METH conditioned
Dong-Liang Jiao, Dong-Liang Jiao, Yan Chen, Yan Chen, Yao Liu, Yun-Yue Ju, Jian-Dong Long, Jiang Du, Chang-Xi Yu, Yu-Jun Wang, Min Zhao, Min Zhao, Jing-Gen Liu   +12 more
doaj   +1 more source

Targeting CD300a Signaling With a Phosphatidylserine‐Presenting Hydrogel Rescues Macrophage Dysfunction and Accelerates Diabetic Wound Healing

Advanced Healthcare Materials, EarlyView.
Schematic illustration of the proposed mechanism: PEG/RGD‐PSLs mimic apoptotic cells to engage PS receptors (notably CD300a), transducing an inhibitory signal that suppresses the MyD88/NF‐κB pathway, leading to global anti‐inflammatory and pro‐reparative effects.
Lele Wu, Xuetong Hao, Yang Zou, Xinglin Chen, Junyan Kong, Chunning Gu, Wenjie Ma, Zhongjun Yang, Huifen Liu, Mengmeng Liu, Xin Tong   +10 more
wiley   +1 more source

Dexamethasone-induced selenoprotein S degradation is required for adipogenesis

Journal of Lipid Research, 2013
Although adipogenesis is associated with induction of endoplasmic reticulum (ER) stress, the role of selenoprotein S (SEPS1), an ER resident selenoprotein known to regulate ER stress and ER-associated protein degradation, is unknown.
Choon Young Kim, Kee-Hong Kim
doaj   +1 more source

Comprehensive In Vitro Profiling Demonstrates Successful Immune Evasion of Bioengineered Immune‐Shielded Heart Valves

Advanced Healthcare Materials, EarlyView.
Living donor heart valves offer superior durability for pediatric patients but trigger immune rejection. Lentiviral delivery of viral immune‐evasion genes US2 and Serpin b9 into donor valve cells and valve tissue suppresses HLA expression, reduces PBMC clustering and cytotoxic killing, and induces transcriptional immune‐tolerance programs, paving the ...
Abraham van Wijk, Leanne Van den Berg, Isa B. M. Velraeds, Valentina Osorio Zuluaga, Emma T. M. Peereboom, Martijn J. W. E. Rabelink, Arnaud Zaldumbide, Rob C. Hoeben, Marie‐Jose T. H. Goumans, Eric Spierings, Carlijn V. C. Bouten, Marijn C. Peters   +11 more
wiley   +1 more source

Sustainable Poly(Lactic Acid)/Graphene Oxide Bioelectronic Platform for Neurotransmitters Sensing and Tunable Stimulation of Astrocytes

Advanced Materials, EarlyView.
This work introduces sustainable PLA/graphene oxide bioelectronic interfaces featuring electrodes with tunable conductivity and strong electrocatalytic performance. These platforms were used to implement a new method for tuning astrocyte Ca2+ signaling and for the efficient detection of key biomarkers.
Alessandra Scidà, Giulia Cazzador, Roberta Fabbri, Matteo Calosi, Alessandro Silvestri, Andrea Candini, Fabiola Liscio, Franco Corticelli, Aikaterini Konstantoulaki, Andrea D'Iorio, Marco Caprini, Vincenzo Palermo, Monica Bertoldo, Valentina Benfenati, Chiara Zanardi, Emanuele Treossi   +15 more
wiley   +1 more source