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Sotaro Naoi et al.
The Journal of pediatrics, 164(5), 1219-1227 (2014-02-18)
To examine the effects of 4-phenylbutyrate (4PB) therapy in a patient with progressive familial intrahepatic cholestasis type 2. A homozygous c.3692G>A (p.R1231Q) mutation was identified in ABCB11. In vitro studies showed that this mutation decreased the cell-surface expression of bile
David W Kamp et al.
American journal of respiratory cell and molecular biology, 49(6), 892-901 (2013-07-28)
Asbestos exposure results in pulmonary fibrosis (asbestosis) and malignancies (bronchogenic lung cancer and mesothelioma) by mechanisms that are not fully understood. Alveolar epithelial cell (AEC) apoptosis is important in the development of pulmonary fibrosis after exposure to an array of
Stephen Freeman et al.
EMBO reports, 20(9), e47097-e47097 (2019-07-20)
Protein homeostasis is essential to cell function, and a compromised ability to reduce the load of misfolded and aggregated proteins is linked to numerous age-related diseases, including hearing loss. Here, we show that altered proteostasis consequent to Elongator complex deficiency
Zhejun Cai et al.
Arteriosclerosis, thrombosis, and vascular biology, 33(10), 2345-2354 (2013-08-10)
Aortic valve (AV) calcification occurs via a pathophysiological process that includes lipoprotein deposition, inflammation, and osteoblastic differentiation of valvular interstitial cells. Here, we investigated the association between endoplasmic reticulum (ER) stress and AV calcification. We identified ER stress activation in
Vanessa Dubois et al.
Molecular systems biology, 16(5), e9156-e9156 (2020-05-15)
Liver injury triggers adaptive remodeling of the hepatic transcriptome for repair/regeneration. We demonstrate that this involves particularly profound transcriptomic alterations where acute induction of genes involved in handling of endoplasmic reticulum stress (ERS) is accompanied by partial hepatic dedifferentiation. Importantly
Viola Pomozi et al.
The Journal of investigative dermatology, 134(4), 946-953 (2013-12-20)
Mutations in the ABCC6 gene cause soft-tissue calcification in pseudoxanthoma elasticum (PXE) and, in some patients, generalized arterial calcification of infancy (GACI). PXE is characterized by late onset and progressive mineralization of elastic fibers in dermal, ocular, and cardiovascular tissues.
Alba Corman et al.
Cell chemical biology, 26(2), 235-243 (2018-12-12)
The expansion of GGGGCC repeats within the first intron of C9ORF72 constitutes the most common cause of amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD). Through repeat-associated non-ATG translation, these expansions are translated into dipeptide repeats (DPRs), some of which
The Molecular and Genetic Basis of Neurologic and Psychiatric Disease (2008)
Elisabeth Kemter et al.
The Journal of biological chemistry, 289(15), 10715-10726 (2014-02-26)
Uromodulin (UMOD)-associated kidney disease (UAKD) belongs to the hereditary progressive ER storage diseases caused by maturation defects of mutant UMOD protein. Current treatments of UAKD patients are symptomatic and cannot prevent disease progression. Two in vitro studies reported a positive
Advances in Clinical Chemistry, Volume 17 (2014)
Eun Sang Lee et al.
Biochemical and biophysical research communications, 450(2), 1005-1009 (2014-06-29)
Radiotherapy, which is one of the most effective approaches to the treatment of various cancers, plays an important role in malignant cell eradication in the pelvic area and abdomen. However, it also generates some degree of intestinal injury. Apoptosis in
Tao Luo et al.
Chemico-biological interactions, 225, 90-98 (2014-12-03)
Endoplasmic reticulum (ER) stress is implicated in the pathophysiology of various cardiovascular diseases, but the role of ER stress in cardiac rupture and/or remodeling after myocardial infarction (MI) is still unclear. Here we investigated whether ER stress plays a major
Xiujing Dou et al.
Food & nutrition research, 62 (2018-12-24)
Host defense peptides (HDPs) possess direct antibacterial, antineoplastic, and immunomodulatory abilities, playing a vital role in innate immunity. Dietary-regulated HDP holds immense potential as a novel pathway for preventing infection. In this study, we examined the regulation mechanism of HDPs
Ruoxuan Xiao et al.
Cancer letters, 475, 22-33 (2020-02-06)
Non-small cell lung cancer (NSCLC) is one of the leading causes of cancer-related mortality. Artemisinin (ART) and SOMCL-14-221 (221), a spirobicyclic analogue of ART, have been reported to inhibit the proliferation of A549 cells with unclear underlying mechanism. In the present
Nikhil N Kulkarni et al.
Molecular immunology, 63(2), 530-539 (2014-12-03)
Antimicrobial peptides (AMPs) constitute an indispensable arm of innate immunity against infectious microbes in humans. Induction of endogenous AMPs may become an alternative therapy against infections. Our previous studies have demonstrated phenylbutyrate (PBA) as a novel inducer of the AMPs
Gulab S Zode et al.
The Journal of clinical investigation, 124(5), 1956-1965 (2014-04-03)
Administration of glucocorticoids induces ocular hypertension in some patients. If untreated, these patients can develop a secondary glaucoma that resembles primary open-angle glaucoma (POAG). The underlying pathology of glucocorticoid-induced glaucoma is not fully understood, due in part to lack of
Kyoungsub Song et al.
Hepatology (Baltimore, Md.), 58(5), 1681-1692 (2013-05-25)
MicroRNA-122 (miR-122), a pivotal liver-specific miRNA, has been implicated in several liver diseases including hepatocellular carcinoma (HCC) and hepatitis C and B viral infection. This study aimed to explore epigenetic regulation of miR-122 in human HCC cells and to examine
Phase 2 comparison of a novel ammonia scavenging agent with sodium phenylbutyrate in patients with urea cycle disorders: safety, pharmacokinetics and ammonia control
Lee B, et al.
Molecular Genetics and Metabolism, 100(3), 221-228 (2010)
Wei Zeng et al.
Life sciences, 103(1), 15-24 (2014-03-22)
Endoplasmic reticulum (ER) stress is involved in the pathogenesis of atherosclerosis (AS). Endothelial cell (EC) dysfunction and monocyte migration to the subendothelium are considered to be essential manifestations of AS. We conducted this study to determine whether ER stress was
Ah Young Lee et al.
Food and chemical toxicology : an international journal published for the British Industrial Biological Research Association, 83, 183-192 (2015-06-28)
In this study, we examined the mechanism underlying the effect of Saururus chinensis Baill (saururaceae) on hepatocellular carcinoma HepG2 cells. HepG2 cells and Chang cells were exposed to various concentrations of S. chinensis Baill extract (SC-E) for 24 h. SC-E
Hematopoietic stimulation by amifostine and sodium phenylbutyrate: what is the potential in MDS?
A F List
Leukemia research, 22 Suppl 1, S7-11 (1998-09-12)
Ziyi Wu et al.
Frontiers in behavioral neuroscience, 15, 632143-632143 (2021-03-02)
Neonatal hypoxic-ischemic (HI) brain injury is associated with long-term neurological disorders, and protective strategies are presently scarce. Sodium 4-phenylbutyrate (4-PBA) reportedly acts as a chemical chaperone that alleviates endoplasmic reticulum (ER) stress, which plays a critical role in neurological diseases.
Nelson M LaMarche et al.
Cell metabolism, 32(2), 243-258 (2020-06-10)
Adipose tissue invariant natural killer T (iNKT) cells are phenotypically different from other iNKT cells because they produce IL-10 and control metabolic homeostasis. Why that is the case is unclear. Here, using single-cell RNA sequencing, we found several adipose iNKT
Ross M Tanis et al.
Biochimica et biophysica acta, 1853(1), 213-221 (2014-12-03)
While the 3T3-L1 adipocyte model is routinely used for the study of obesity and diabetes, the mitochondrial respiratory profile in normal versus high glucose has not been examined in detail. We matured adipocytes in normal (5mM) or high (30 mM)
Association between betatrophin/ANGPTL8 and non-alcoholic fatty liver disease: animal and human studies
Lee YH, et al.
Scientific Reports, 6(3), 24013-24013 (2016)
Yuyuan Liu et al.
Molecular medicine reports, 20(1), 755-762 (2019-06-11)
The present study investigated the role of the c‑Jun N‑terminal kinase (JNK)/transforming growth factor‑β (TGF‑β)/Smad3 pathway in endoplasmic reticulum stress (ERS)‑mediated renal interstitial fibrosis, which would be beneficial for chronic kidney disease (CKD) therapy. In human renal biopsy tissue, the
Norihiko Yokoi et al.
Nature medicine, 21(1), 19-26 (2014-12-09)
Epilepsy is one of the most common and intractable brain disorders. Mutations in the human gene LGI1, encoding a neuronal secreted protein, cause autosomal dominant lateral temporal lobe epilepsy (ADLTE). However, the pathogenic mechanisms of LGI1 mutations remain unclear. We
Hilaire C Lam et al.
The Journal of clinical investigation, 123(12), 5212-5230 (2013-11-10)
Chronic obstructive pulmonary disease (COPD) involves aberrant airway inflammatory responses to cigarette smoke (CS) that are associated with epithelial cell dysfunction, cilia shortening, and mucociliary clearance disruption. Exposure to CS reduced cilia length and induced autophagy in vivo and in
Khadija Syeda et al.
Biochemical pharmacology, 86(9), 1338-1346 (2013-09-03)
Nuclear lamins form the lamina on the interior of the nuclear envelope, and are involved in the regulation of various cellular processes, including DNA replication and chromatin organization. Despite this evidence, little is known about potential alterations in nuclear metabolism
Masayuki Koyama et al.
American journal of physiology. Heart and circulatory physiology, 306(9), H1314-H1323 (2014-03-13)
Pulmonary arterial hypertension (PAH) is characterized by vasoconstriction and vascular remodeling of the pulmonary artery (PA). Recently, endoplasmic reticulum (ER) stress and inappropriate adaptation through the unfolded protein response (UPR) have been disclosed in various types of diseases. Here we
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