Relationship Between Neurologic Symptoms and Signs and FMR1 Genotype in Premutation Carriers
ABSTRACT Background and Objectives Fragile X‐associated Tremor/Ataxia Syndrome (FXTAS) is the most severe late‐onset condition caused by a premutation in the FMR1 gene, characterized by expanded CGG triplet repeats of 55–200. Clinical presentations of FXTAS, including gait ataxia, kinetic tremor, cognitive decline, and rare Parkinsonism, are linked to ...
Flora Tassone +8 more
wiley +1 more source
CDC123 is an ATPase that modulates mRNA translation and the integrated stress response by regulating eIF2 complex assembly. [PDF]
Erb AL, Young-Baird SK.
europepmc +1 more source
Emerging roles for integrated stress response signaling in homeostasis. [PDF]
Nandakumar S, Grmai L, Vasudevan D.
europepmc +1 more source
SARS-CoV-2 and MERS-CoV disrupt host protein synthesis via nsp1 with differential effects on the integrated stress response. [PDF]
Parenti NA +11 more
europepmc +1 more source
Inhibition of the integrated stress response rescues a histidyl-tRNA synthetase variant associated with Charcot-Marie-Tooth disease. [PDF]
Mahmood M +8 more
europepmc +1 more source
Pharmacological targeting of RIG-I can selectively activate the integrated stress response. [PDF]
Cuoco CA +23 more
europepmc +1 more source
The integrated stress response [PDF]
In response to diverse stress stimuli, eukaryotic cells activate a common adaptive pathway, termed the integrated stress response (ISR), to restore cellular homeostasis.
Izabela Koryga +2 more
exaly +6 more sources
The Integrated Stress Response in Lung Disease [PDF]
Lungs are repeatedly exposed to inhaled toxic insults, such as smoke, diesel exhaust, and microbes, which elicit cellular stress responses. The phosphorylation of eukaryotic translation initiation factor 2α by one of four stress-sensing kinases triggers ...
Stefan Marciniak, Pieter Hiemstra
exaly +7 more sources
Folding the Mitochondrial UPR into the Integrated Stress Response
Eukaryotic cells must accurately monitor the integrity of the mitochondrial network to overcome environmental insults and respond to physiological cues. The mitochondrial unfolded protein response (UPRmt) is a mitochondrial-to-nuclear signaling pathway that maintains mitochondrial proteostasis, mediates signaling between tissues, and regulates ...
Cole Haynes
exaly +4 more sources
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Surviving and Adapting to Stress: Translational Control and the Integrated Stress Response
Antioxidants and Redox Signaling, 2023Significance: Organisms adapt to changing environments by engaging cellular stress response pathways that serve to restore proteostasis and enhance survival. A primary adaptive mechanism is the integrated stress response (ISR), which features phosphorylation of the α subunit of eukaryotic translation initiation factor 2 (eIF2).
Kirk Staschke +2 more
exaly +3 more sources

