Abstract:
:(1) Background: A high incidence of intervening sequence (IVS)4+919 G>A mutation with later-onset cardiac phenotype have been reported in a majority of Taiwan Fabry cohorts. Some evidence indicated that conventional biomarkers failed to predict the long-term progression and therapeutic outcome; (2) Methods: In this study, we constructed an induced pluripotent stem cell (iPSC)-based platform from Fabry cardiomyopathy (FC) patients carrying IVS4+919 G>A mutation to screen for potential targets that may help the conventional treatment; (3) Results: The FC-patient-derived iPSC-differentiated cardiomyocytes (FC-iPSC-CMs) carried an expected IVS4+919 G>A genetic mutation and recapitulated several FC characteristics, including low α-galactosidase A enzyme activity and cellular hypertrophy. The proteomic analysis revealed that arachidonate 12/15-lipoxygenase (Alox12/15) was the most highly upregulated marker in FC-iPSC-CMs, and the metabolites of Alox12/15, 12(S)- and 15(S)-hydroxyeicosatetraenoic acid (HETE), were also elevated in the culture media. Late administration of Alox12/15 pharmacological inhibitor LOXBlock-1 combined with α-galactosidase, but not α-galactosidase alone, effectively reduced cardiomyocyte hypertrophy, the secretion of 12(S)- and 15(S)-HETE and the upregulation of fibrotic markers at the late phase of FC; (4) Conclusions: Our study demonstrates that cardiac Alox12/15 and circulating 12(S)-HETE/15(S)-HETE are involved in the pathogenesis of FC with IVS4+919 G>A mutation.
journal_name
Int J Mol Scijournal_title
International journal of molecular sciencesauthors
Chien Y,Chou SJ,Chang YL,Leu HB,Yang YP,Tsai PH,Lai YH,Chen KH,Chang WC,Sung SH,Yu WCdoi
10.3390/ijms19051480subject
Has Abstractpub_date
2018-05-16 00:00:00issue
5issn
1422-0067pii
ijms19051480journal_volume
19pub_type
杂志文章abstract::The Bromeliaceae family is one of the most morphologically diverse families with a pantropical distribution. To schedule an appropriate flowering time for bromeliads, ethylene is commonly used to initiate flower development in adult plants. However, the mechanism by which ethylene induces flowering in adult bromeliads...
journal_title:International journal of molecular sciences
pub_type: 杂志文章
doi:10.3390/ijms17030303
更新日期:2016-02-27 00:00:00
abstract::The onset and selection of resistant clones during cancer treatment with chemotherapy or targeted therapy is a major issue in the clinical management of metastatic colorectal cancer patients. It is possible that a more personalized treatment selection, using reliable response-to-therapy predictive biomarkers, could le...
journal_title:International journal of molecular sciences
pub_type: 杂志文章,评审
doi:10.3390/ijms18071547
更新日期:2017-07-17 00:00:00
abstract::RNAi technology has significant potential as a future therapeutic and could theoretically be used to knock down disease-specific RNAs. However, due to frequent off-target effects, low efficiency, and limited accessibility of nuclear transcripts, the clinical application of the technology remains challenging. In this s...
journal_title:International journal of molecular sciences
pub_type: 杂志文章
doi:10.3390/ijms21238904
更新日期:2020-11-24 00:00:00
abstract::Aging is a major risk factor of osteoarthritis, which is characterized by the degeneration of articular cartilage. CCN3, a member of the CCN family, is expressed in cartilage and has various physiological functions during chondrocyte development, differentiation, and regeneration. Here, we examine the role of CCN3 in ...
journal_title:International journal of molecular sciences
pub_type: 杂志文章
doi:10.3390/ijms21207556
更新日期:2020-10-13 00:00:00
abstract::STIM1 is an endoplasmic reticulum (ER) protein that modulates the activity of a number of Ca2+ transport systems. By direct physical interaction with ORAI1, a plasma membrane Ca2+ channel, STIM1 activates the ICRAC current, whereas the binding with the voltage-operated Ca2+ channel CaV1.2 inhibits the current through ...
journal_title:International journal of molecular sciences
pub_type: 杂志文章
doi:10.3390/ijms21186598
更新日期:2020-09-09 00:00:00
abstract::The effects of various inhibitors on crude, commercial and partially purified commercial mushroom tyrosinase were examined by comparing IC(50) values. Kojic acid, salicylhydroxamic acid, tropolone, methimazole, and ammonium tetrathiomolybdate had relatively similar IC(50) values for the crude, commercial and partially...
journal_title:International journal of molecular sciences
pub_type: 杂志文章
doi:10.3390/ijms10093811
更新日期:2009-09-02 00:00:00
abstract::In this paper, we investigated the activation energies of the aggregation−disaggregation self-oscillation induced by the Belousov-Zhabotinsky (BZ) reaction by utilizing the nonthermoresponsive polymer chain in a wide temperature range. This is because the conventional type self-oscillating polymer chain, with thermore...
journal_title:International journal of molecular sciences
pub_type: 杂志文章
doi:10.3390/ijms131216281
更新日期:2012-12-03 00:00:00
abstract:BACKGROUND:Psoriasis is hallmarked by vascular dysfunction, arterial hypertension, and an increased risk for cardiovascular diseases. We have shown recently that skin-driven interleukin (IL)-17A expression promotes psoriasis-like disease in mice, and this is associated with vascular inflammation, vascular dysfunction, ...
journal_title:International journal of molecular sciences
pub_type: 杂志文章
doi:10.3390/ijms20174261
更新日期:2019-08-30 00:00:00
abstract::The micro RNA (miR)-34 family is composed of 5p and 3p strands of miR-34a, miR-34b, and miR-34c. The 5p strand's expression and function is studied in cervical cancer. The 3p strand's function and regulation remain to be elucidated. To study the function of the passenger strands of miR-34 family members, we overexpres...
journal_title:International journal of molecular sciences
pub_type: 杂志文章
doi:10.3390/ijms20030545
更新日期:2019-01-28 00:00:00
abstract::Articular cartilage is a structure lack of vascular distribution. Once the cartilage is injured or diseased, it is unable to regenerate by itself. Surgical treatments do not effectively heal defects in articular cartilage. Tissue engineering is the most potential solution to this problem. In this study, methoxy poly(e...
journal_title:International journal of molecular sciences
pub_type: 杂志文章
doi:10.3390/ijms19041125
更新日期:2018-04-09 00:00:00
abstract::The relationship between antiphospholipid antibodies (aPL) and autoimmune haemolytic anaemia (AIHA) has never been systematically addressed. The aim of this study is to assess the link between aPL and AIHA in adult systemic lupus erythematosus (SLE) and antiphospholipid syndrome (APS). This study performed an EMBASE/P...
journal_title:International journal of molecular sciences
pub_type: 杂志文章,评审
doi:10.3390/ijms21114120
更新日期:2020-06-09 00:00:00
abstract::In the last several years, researchers have exhibited an intense interest in the evolutionarily conserved signaling pathways that have crucial roles during embryonic development. Interestingly, the malfunctioning of these signaling pathways leads to several human diseases, including cancer. The chemical and biophysica...
journal_title:International journal of molecular sciences
pub_type: 杂志文章,评审
doi:10.3390/ijms13066561
更新日期:2012-01-01 00:00:00
abstract::Acyl-CoA:glycerol-sn-3-phosphate acyltransferase (GPAT) is an enzyme responsible for the rate-limiting step in the synthesis of glycerophospholipids and triacylglycerol (TAG). The enzymes of mammalian species are classified into four isoforms; GPAT1 and GPAT2 are localized in the mitochondrial outer membrane, whereas ...
journal_title:International journal of molecular sciences
pub_type: 杂志文章,评审
doi:10.3390/ijms20040964
更新日期:2019-02-22 00:00:00
abstract::The hyperthermophilic archaeon Thermococcus onnurineus NA1 has been shown to produce H₂ when using CO, formate, or starch as a growth substrate. This strain can also utilize elemental sulfur as a terminal electron acceptor for heterotrophic growth. To gain insight into sulfur metabolism, the proteome of T. onnurineus ...
journal_title:International journal of molecular sciences
pub_type: 杂志文章
doi:10.3390/ijms16059167
更新日期:2015-04-23 00:00:00
abstract::With a global incidence of 1.8 million cases, colorectal cancer represents one of the most common cancers worldwide. Despite impressive improvements in treatment efficacy through cytotoxic and biological agents, the cancer-related death burden of metastatic colorectal cancer (mCRC) is still high. mCRC is not a genetic...
journal_title:International journal of molecular sciences
pub_type: 杂志文章,评审
doi:10.3390/ijms21239001
更新日期:2020-11-26 00:00:00
abstract::The juvenile hormone (JH) signalling and ecdysone signalling pathways are crucial endocrine signalling pathways that orchestrate the metamorphosis of insects. The metamorphic process, the morphological change from the immature to adult forms, is orchestrated by the dramatic reduction of JH and downstream transcription...
journal_title:International journal of molecular sciences
pub_type: 杂志文章
doi:10.3390/ijms20102431
更新日期:2019-05-16 00:00:00
abstract::Gaucher disease (GD) is an autosomal recessive lysosomal storage disorder caused by mutations in the acid β-glucosidase gene (GBA1). Besides causing GD, GBA1 mutations constitute the main genetic risk factor for developing Parkinson's disease. The molecular basis of neurological manifestations in GD remain elusive. Ho...
journal_title:International journal of molecular sciences
pub_type: 杂志文章
doi:10.3390/ijms21093268
更新日期:2020-05-05 00:00:00
abstract::Characterizations of fatty acids composition in % of total methylester of fatty acids (FAMEs) of fourteen vegetable oils--safflower, grape, silybum marianum, hemp, sunflower, wheat germ, pumpkin seed, sesame, rice bran, almond, rapeseed, peanut, olive, and coconut oil--were obtained by using gas chromatography (GC). S...
journal_title:International journal of molecular sciences
pub_type: 杂志文章
doi:10.3390/ijms160612871
更新日期:2015-06-05 00:00:00
abstract::Accurate prediction of the potential hepatotoxic nature of new pharmaceuticals remains highly challenging. Therefore, novel in vitro models with improved external validity are needed to investigate hepatic metabolism and timely identify any toxicity of drugs in humans. In this study, we examined the effects of diclofe...
journal_title:International journal of molecular sciences
pub_type: 杂志文章
doi:10.3390/ijms17040584
更新日期:2016-04-16 00:00:00
abstract::Sensitive molecular assays are critical for coronavirus disease 2019 (COVID-19) diagnosis. Here, we designed and evaluated two single-tube nested (STN) real-time RT-PCR assays, targeting SARS-CoV-2 RdRp/Hel and N genes. Both STN assays had a low limit of detection and did not cross react with other human coronaviruses...
journal_title:International journal of molecular sciences
pub_type: 杂志文章
doi:10.3390/ijms21165674
更新日期:2020-08-07 00:00:00
abstract::Trichoderma reesei is a biotechnologically important filamentous fungus with the remarkable ability to secrete large amounts of enzymes, whose production is strongly affected by both the carbon and nitrogen sources. While the carbon metabolism regulators are extensively studied, the regulation of enzyme production by ...
journal_title:International journal of molecular sciences
pub_type: 杂志文章
doi:10.3390/ijms20174100
更新日期:2019-08-22 00:00:00
abstract::Docking-i.e., interaction of a small molecule (ligand) with a proteic structure (receptor)-represents the ground of drug action mechanism of the vast majority of bioactive chemicals. Ligand and receptor accommodate their geometry and energy, within this interaction, in the benefit of receptor-ligand complex. In an ind...
journal_title:International journal of molecular sciences
pub_type: 杂志文章
doi:10.3390/ijms18071377
更新日期:2017-06-27 00:00:00
abstract::Parkinson's disease (PD) is one of the most common neurodegenerative disorders. The neuropathological features of PD are selective and progressive loss of dopaminergic neurons in the substantia nigra pars compacta, deficiencies in striatal dopamine levels, and the presence of intracellular Lewy bodies. Interactions am...
journal_title:International journal of molecular sciences
pub_type: 杂志文章,评审
doi:10.3390/ijms21197182
更新日期:2020-09-29 00:00:00
abstract::Resveratrol exhibits not only anti-melanogenic property by inhibiting microphthalmia-associated transcription factor (MITF), but also anti-aging property by activating sirtuin-1 (SIRT1). In this study, the relationship between depigmenting effect of resveratrol and SIRT1/forkhead box O (FOXO) 3a activation and was inv...
journal_title:International journal of molecular sciences
pub_type: 杂志文章
doi:10.3390/ijms18061213
更新日期:2017-06-07 00:00:00
abstract::Myotonic dystrophy type 1 (DM1) is a complex neuromuscular disease caused by an unstable cardiotocography (CTG) repeat expansion in the DMPK gene. This disease is characterized by high clinical and genetic variability, leading to some difficulties in the diagnosis and prognosis of DM1. Better understanding the origin ...
journal_title:International journal of molecular sciences
pub_type: 杂志文章,评审
doi:10.3390/ijms21020457
更新日期:2020-01-10 00:00:00
abstract::Although mitochondrial dysfunction has been implicated in aging, physical function decline, and several age-related diseases, an accessible and affordable measure of mitochondrial health is still lacking. In this study we identified the proteomic signature of muscular mitochondrial oxidative capacity in plasma. In 165...
journal_title:International journal of molecular sciences
pub_type: 杂志文章
doi:10.3390/ijms21249540
更新日期:2020-12-15 00:00:00
abstract::Histone H1 is the most variable histone and its role at the epigenetic level is less characterized than that of core histones. In vertebrates, H1 is a multigene family, which can encode up to 11 subtypes. The H1 subtype composition is different among cell types during the cell cycle and differentiation. Mass spectrome...
journal_title:International journal of molecular sciences
pub_type: 杂志文章,评审
doi:10.3390/ijms21165941
更新日期:2020-08-18 00:00:00
abstract::To identify whether parent-of-origin effects (POE) of the 15q11.2 BP1-BP2 microdeletion are associated with differences in clinical features in individuals inheriting the deletion, we collected 71 individuals reported with phenotypic data and known inheritance from a clinical cohort, a research cohort, the DECIPHER da...
journal_title:International journal of molecular sciences
pub_type: 杂志文章
doi:10.3390/ijms20061459
更新日期:2019-03-22 00:00:00
abstract::There are still many challenges to acquire the optimal integration of biomedical materials with the surrounding tissues. Gene coatings on the surface of biomaterials may offer an effective approach to solve the problem. In order to investigate the gene multilayers mediated differentiation of mesenchymal stem cells (MS...
journal_title:International journal of molecular sciences
pub_type: 杂志文章
doi:10.3390/ijms151120117
更新日期:2014-11-05 00:00:00
abstract::Our previous study indicated that the combination of salvianolic acid B (SalB) and ginsenoside Rg1 (Rg1), the main components of Salvia miltiorrhizae and Panax notoginseng, improves myocardium structure and ventricular function in rats with ischemia/reperfusion injury. The present study aimed to determine the safety o...
journal_title:International journal of molecular sciences
pub_type: 杂志文章
doi:10.3390/ijms161226176
更新日期:2015-12-09 00:00:00