The therapeutic effects of 4-phenylbutyric acid in maintaining proteostasis
Recently, there has been an increasing amount of literature published on the effects of 4-
phenylbutyric acid (4-PBA) in various biological systems. 4-PBA is currently used clinically to …
phenylbutyric acid (4-PBA) in various biological systems. 4-PBA is currently used clinically to …
COL4A1 and COL4A2 mutations and disease: insights into pathogenic mechanisms and potential therapeutic targets
DS Kuo, C Labelle-Dumais… - Human molecular …, 2012 - academic.oup.com
Heterotrimers composed of collagen type IV alpha 1 (COL4A1) and alpha 2 (COL4A2)
constitute one of the most abundant components of nearly all basement membranes …
constitute one of the most abundant components of nearly all basement membranes …
[HTML][HTML] Treatment of obesity with celastrol
Despite all modern advances in medicine, an effective drug treatment of obesity has not
been found yet. Discovery of leptin two decades ago created hopes for treatment of obesity …
been found yet. Discovery of leptin two decades ago created hopes for treatment of obesity …
Aggregation-prone c9FTD/ALS poly (GA) RAN-translated proteins cause neurotoxicity by inducing ER stress
YJ Zhang, K Jansen-West, YF Xu, TF Gendron… - Acta …, 2014 - Springer
The occurrence of repeat-associated non-ATG (RAN) translation, an atypical form of
translation of expanded repeats that results in the synthesis of homopolymeric expansion …
translation of expanded repeats that results in the synthesis of homopolymeric expansion …
The therapeutic potential of chemical chaperones in protein folding diseases
Several neurodegenerative diseases are caused by defects in protein folding, including
Alzheimer, Parkinson, Huntington, and prion diseases. Once a disease-specific protein …
Alzheimer, Parkinson, Huntington, and prion diseases. Once a disease-specific protein …
Strategies for treating idiopathic pulmonary fibrosis
RM Du Bois - Nature reviews Drug discovery, 2010 - nature.com
Idiopathic pulmonary fibrosis (IPF) is the most common and most lethal diffuse fibrosing lung
disease, with a mortality rate that exceeds that of many cancers. Recently, there have been …
disease, with a mortality rate that exceeds that of many cancers. Recently, there have been …
[HTML][HTML] Activated ATF6 induces intestinal dysbiosis and innate immune response to promote colorectal tumorigenesis
OI Coleman, EM Lobner, S Bierwirth, A Sorbie… - Gastroenterology, 2018 - Elsevier
Background & Aims Activating transcription factor 6 (ATF6) regulates endoplasmic reticulum
stress. We studied whether ATF6 contributes to the development of colorectal cancer (CRC) …
stress. We studied whether ATF6 contributes to the development of colorectal cancer (CRC) …
β-cell dysfunction due to increased ER stress in a stem cell model of Wolfram syndrome
L Shang, H Hua, K Foo, H Martinez, K Watanabe… - Diabetes, 2014 - Am Diabetes Assoc
Wolfram syndrome is an autosomal recessive disorder caused by mutations in WFS1 and is
characterized by insulin-dependent diabetes mellitus, optic atrophy, and deafness. To …
characterized by insulin-dependent diabetes mellitus, optic atrophy, and deafness. To …
Endoplasmic reticulum stress in obesity and obesity-related disorders: An expanded view
MJ Pagliassotti, PY Kim, AL Estrada, CM Stewart… - Metabolism, 2016 - Elsevier
The endoplasmic reticulum (ER) is most notable for its central roles in calcium ion storage,
lipid biosynthesis, and protein sorting and processing. By virtue of its extensive membrane …
lipid biosynthesis, and protein sorting and processing. By virtue of its extensive membrane …
The chemical chaperone 4-phenylbutyrate inhibits adipogenesis by modulating the unfolded protein response [S]
Recent studies have shown a link between obesity and endoplasmic reticulum (ER) stress.
Perturbations in ER homeostasis cause ER stress and activation of the unfolded protein …
Perturbations in ER homeostasis cause ER stress and activation of the unfolded protein …