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https://www.readbyqxmd.com/read/28723748/clinical-characteristics-and-mutation-spectrum-of-gla-in-korean-patients-with-fabry-disease-by-a-nationwide-survey-underdiagnosis-of-late-onset-phenotype
#1
Jin-Ho Choi, Beom Hee Lee, Sun Hee Heo, Gu-Hwan Kim, Yoo-Mi Kim, Dae-Seong Kim, Jung Min Ko, Young Bae Sohn, Yong Hee Hong, Dong-Hwan Lee, Hoon Kook, Han Hyuk Lim, Kyung Hee Kim, Woo-Shik Kim, Geu-Ru Hong, Su-Hyun Kim, Sang Hyun Park, Chan-Duck Kim, So Mi Kim, Jeong-Sook Seo, Han-Wook Yoo
Fabry disease is a rare X-linked lysosomal storage disorder caused by an α-galactosidase A deficiency. The progressive accumulation of globotriaosylceramide (GL-3) results in life-threatening complications, including renal, cardiac, and cerebrovascular diseases. This study investigated the phenotypic and molecular spectra of GLA mutations in Korean patients with Fabry disease using a nationwide survey.This study included 94 patients from 46 independent pedigrees: 38 adult males, 46 symptomatic females, and 10 pediatric males...
July 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28720775/metformin-ameliorates-the-phenotype-transition-of-peritoneal-mesothelial-cells-and-peritoneal-fibrosis-via-a-modulation-of-oxidative-stress
#2
Hyun-Soo Shin, Jiyeon Ko, Dal-Ah Kim, Eun-Sun Ryu, Hye-Myung Ryu, Sun-Hee Park, Yong-Lim Kim, Eok-Soo Oh, Duk-Hee Kang
Phenotype transition of peritoneum is an early mechanism of peritoneal fibrosis. Metformin, 5'-adenosine monophosphate-activated protein kinase (AMPK) activator, has recently received a new attention due to its preventive effect on organ fibrosis and cancer metastasis by inhibiting epithelial-to-mesenchymal transition (EMT). We investigated the effect of metformin on EMT of human peritoneal mesothelial cells (HPMC) and animal model of peritoneal dialysis (PD). TGF-β1-induced EMT in HPMC was ameliorated by metformin...
July 18, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28717651/comparison-of-nonspherical-polyvinyl-alcohol-particles-and-microspheres-for-prostatic-arterial-embolization-in-patients-with-benign-prostatic-hyperplasia
#3
Jin Ho Hwang, Sang Woo Park, Il Soo Chang, Sung Il Jung, Hae Jeong Jeon, Yong Soo Lho, Hyeong Gon Kim, Sung Hyun Paick, Hyoung Keun Park
PURPOSE: To report early results following prostatic artery embolization (PAE) and compare outcomes between nonspherical polyvinyl alcohol (PVA) particles and microspheres to treat lower urinary tract symptoms (LUTS) due to benign prostatic hyperplasia (BPH). METHODS: PAE was performed in nine patients (mean age: 78.1 years) with symptomatic BPH. Embolization was performed using nonspherical PVA particles (250-355 μm) in four patients and microspheres (300-500 μm) in five patients...
2017: BioMed Research International
https://www.readbyqxmd.com/read/28714239/self-powered-real-time-arterial-pulse-monitoring-using-ultrathin-epidermal-piezoelectric-sensors
#4
Dae Yong Park, Daniel J Joe, Dong Hyun Kim, Hyewon Park, Jae Hyun Han, Chang Kyu Jeong, Hyelim Park, Jung Gyu Park, Boyoung Joung, Keon Jae Lee
Continuous monitoring of an arterial pulse using a pressure sensor attached on the epidermis is an important technology for detecting the early onset of cardiovascular disease and assessing personal health status. Conventional pulse sensors have the capability of detecting human biosignals, but have significant drawbacks of power consumption issues that limit sustainable operation of wearable medical devices. Here, a self-powered piezoelectric pulse sensor is demonstrated to enable in vivo measurement of radial/carotid pulse signals in near-surface arteries...
July 17, 2017: Advanced Materials
https://www.readbyqxmd.com/read/28713064/novel-risk-stratification-for-metachronous-recurrence-after-curative-endoscopic-submucosal-dissection-for-early-gastric-cancer
#5
Hyo-Joon Yang, Sang Gyun Kim, Joo Hyun Lim, Ji Min Choi, Sooyeon Oh, Jae Yong Park, Seung Jun Han, Jung Kim, Hyunsoo Chung, Hyun Chae Jung
BACKGROUND AND AIMS: This study stratified the risk of developing metachronous gastric cancer (MGC) after curative endoscopic submucosal dissection (ESD) of early gastric cancer (EGC) to enable the customization of endoscopic surveillance for MGC. METHODS: A total of 1115 patients who underwent curative ESD based on the expanded criteria for differentiated EGC from 2005 to 2014 at a single tertiary hospital were enrolled in this retrospective cohort study. They were followed up with annual endoscopy for a median of 50...
July 13, 2017: Gastrointestinal Endoscopy
https://www.readbyqxmd.com/read/28708407/enhancing-p-type-thermoelectric-performances-of-polycrystalline-snse-via-tuning-phase-transition-temperature
#6
Yong Kyu Lee, Kyunghan Ahn, Joonil Cha, Chongjian Zhou, Hyo Seok Kim, Garam Choi, Sue In Chae, Jae-Hyuk Park, Yeseul Lee, Cheol-Hee Park, Sung-Pyo Cho, Sang Hyun Park, Yung-Eun Sung, Won Bo Lee, Taeghwan Hyeon, In Chung
SnSe emerges as a new class of thermoelectric materials since recent discovery of an ultrahigh thermoelectric figure of merit in its single crystals. Achieving such performance in the polycrystalline counterpart is still challenging and requires fundamental understandings of its electrical and thermal transport properties as well as structural chemistry. Here we demonstrate a new strategy of improving conversion efficiency of bulk polycrystalline SnSe thermoelectrics. We show that PbSe alloying decreases the transition temperature between Pnma and Cmcm phases and thereby can serve as a means of controlling its onset temperature...
July 14, 2017: Journal of the American Chemical Society
https://www.readbyqxmd.com/read/28701722/asxl1-deficiency-in-embryonic-fibroblasts-leads-to-cellular-senescence-via-impairment-of-the-akt-e2f-pathway-and-ezh2-inactivation
#7
Hye Sook Youn, Tae-Yoon Kim, Ui-Hyun Park, Seung-Tae Moon, So-Jung An, Yong-Kyu Lee, Jin-Taek Hwang, Eun-Joo Kim, Soo-Jong Um
Although ASXL1 mutations are frequently found in human diseases, including myeloid leukemia, the cell proliferation-associated function of ASXL1 is largely unknown. Here, we explored the molecular mechanism underlying the growth defect found in Asxl1-deficient mouse embryonic fibroblasts (MEFs). We found that Asxl1, through amino acids 371 to 655, interacts with the kinase domain of AKT1. In Asxl1-null MEFs, IGF-1 was unable to induce AKT1 phosphorylation and activation; p27Kip1, which forms a ternary complex with ASXL1 and AKT1, therefore remained unphosphorylated...
July 12, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28700337/polymorphisms-in-mitotic-checkpoint-related-genes-can-influence-survival-outcomes-of-early-stage-non-small-cell-lung-cancer
#8
Hyo-Gyoung Kang, Seung Soo Yoo, Jin Eun Choi, Mi Jeong Hong, Sook Kyung Do, Cheng Cheng Jin, Soyoun Kim, Won Kee Lee, Sun Ha Choi, So Yeon Lee, Hyun Jung Kim, Shin Yup Lee, Jaehee Lee, Seung Ick Cha, Chang Ho Kim, Yangki Seok, Eungbae Lee, Sukki Cho, Sanghoon Jheon, Jae Yong Park
This study was conducted to investigate the association between variants in mitotic checkpoint-related genes and clinical outcomes of non-small cell lung cancer (NSCLC). A total of 766 patients with NSCLC who underwent curative surgery were enrolled. Among the 73 variants evaluated, 4 variants were related with survival outcomes. BUB3 rs7897156C>T was associated with worse overall survival under a recessive model (adjusted hazard ratio = 1.58, 95% confidence interval = 1.07-2.33, P = 0.02). AURKB rs1059476G>A was associated with better overall survival under a recessive model (adjusted hazard ratio = 0...
June 27, 2017: Oncotarget
https://www.readbyqxmd.com/read/28698865/comparisons-of-gut-microbiota-among-healthy-control-patients-with-conventional-adenoma-sessile-serrated-adenoma-and-colorectal-cancer
#9
Hyuk Yoon, Nayoung Kim, Ji Hyun Park, Yong Sung Kim, Jongchan Lee, Hyoung Woo Kim, Yoon Jin Choi, Cheol Min Shin, Young Soo Park, Dong Ho Lee, Hyun Chae Jung
BACKGROUND: Studies on gut microbiota regarding colorectal carcinogenesis, including sessile serrated adenoma (SSA), have been scarce. The aim of this study is to investigate the role of mucosa-associated gut microbiota in the colorectal carcinogenesis. METHODS: We collected biopsy samples of normal rectal mucosa during colonoscopy from healthy control and patients with conventional adenoma, SSA, and colorectal cancer (CRC), respectively (n = 6). Pyrosequencing for 16S rRNA gene of bacteria was performed to compare gut microbiota...
June 2017: Journal of Cancer Prevention
https://www.readbyqxmd.com/read/28698525/hepatoprotective-role-of-hydrangea-macrophylla-against-sodium-arsenite-induced-mitochondrial-dependent-oxidative-stress-via-the-inhibition-of-mapk-caspase-3-pathways
#10
Md Rashedunnabi Akanda, Hyun-Jin Tae, In-Shik Kim, Dongchoon Ahn, Weishun Tian, Anowarul Islam, Hyeon-Hwa Nam, Byung-Kil Choo, Byung-Yong Park
Sodium arsenite (NaAsO₂) has been recognized as a worldwide health concern. Hydrangea macrophylla (HM) is used as traditional Chinese medicine possessing antioxidant activities. The study was performed to investigate the therapeutic role and underlying molecular mechanism of HM on NaAsO₂-induced toxicity in human liver cancer (HepG2) cells and liver in mice. The hepatoprotective role of HM in HepG2 cells was assessed by using 3-(4,5-dimethylthiazol-2-Yl)-2,5-diphenyltetrazolium bromide (MTT), reactive oxygen species (ROS), and lactate dehydrogenase (LDH) assays...
July 10, 2017: International Journal of Molecular Sciences
https://www.readbyqxmd.com/read/28697599/enhancement-of-friction-by-water-intercalated-between-graphene-and-mica
#11
Hyunsoo Lee, Jae-Hyeon Ko, Jin Sik Choi, Jin Heui Hwang, Yong-Hyun Kim, Miquel B Salmeron, Jeong Young Park
Common experience shows that friction converts mechanical energy into heat. The first part of this process is the vibrational excitation of the atoms at the interface between the rubbing bodies. The second and necessary part is the effective removal of this vibration energy by transfer from the contact region to the bulk substrate. A fundamental understanding of these phenomena is important for the design of improved tribological contact; however, this is difficult because excitation and dissipation are usually intimately entangled processes...
July 11, 2017: Journal of Physical Chemistry Letters
https://www.readbyqxmd.com/read/28692482/prognostic-value-of-brachial-ankle-pulse-wave-velocity-in-patients-with-non-st-elevation-myocardial-infarction
#12
Hyun Woong Park, Min Gyu Kang, Kyehwan Kim, Jin-Sin Koh, Jeong Rang Park, Seok-Jae Hwang, Young-Hoon Jeong, Jong Hwa Ahn, Jeong Yoon Jang, Choong Hwan Kwak, Yongwhi Park, Jin-Yong Hwang
OBJECTIVE: Brachial-ankle pulse wave velocity (baPWV) measurement is a well-established modality for assessing arterial stiffness and predicting cardiovascular events. However, to our knowledge, its usefulness has not been clarified among patients with non-ST-elevation myocardial infarction (NSTEMI). This study assessed the prognostic value of baPWV in patients with NSTEMI. PATIENTS AND METHODS: Patients (n=411, mean age, 63.8±13.5 years, 75.2% men) with NSTEMI who underwent a percutaneous coronary intervention and baPWV measurement were recruited between January 2013 and December 2015...
July 7, 2017: Coronary Artery Disease
https://www.readbyqxmd.com/read/28689995/sirt1-enhances-the-survival-of-human-embryonic-stem-cells-by-promoting-dna-repair
#13
Jiho Jang, Yong Jun Huh, Hyun-Ju Cho, Boram Lee, Jaepil Park, Dong-Youn Hwang, Dong-Wook Kim
Human embryonic stem cells (hESCs) hold great promise for the treatment of many incurable diseases. Sirtuin1 (SIRT1), a class III histone deacetylase, is abundantly expressed in hESCs and is known to regulate early differentiation and telomere elongation. Here, we show that downregulation of SIRT1 promotes cell death in hESCs, but not in differentiated cells, and the SIRT1-inhibition-mediated cell death is preceded by increased DNA damage. This increased DNA damage is at least partially due to decreased levels of DNA repair enzymes such as MSH2, MSH6, and APEX1...
July 5, 2017: Stem Cell Reports
https://www.readbyqxmd.com/read/28689250/multicenter-cross-sectional-observational-study-of-the-impact-of-neuropathic-pain-on-quality-of-life-in-cancer-patients
#14
So Yeon Oh, Sang Won Shin, Su-Jin Koh, Sang Byung Bae, Hyun Chang, Jung Han Kim, Hyo Jung Kim, Young Seon Hong, Keon Uk Park, Jeanno Park, Kyung Hee Lee, Na Ri Lee, Jung Lim Lee, Joung Soon Jang, Dae Sik Hong, Seung-Sei Lee, Sun Kyung Baek, Dae Ro Choi, Jooseop Chung, Sang Cheul Oh, Hye Sook Han, Hwan Jung Yun, Sun Jin Sym, So Young Yoon, In Sil Choi, Byoung Yong Shim, Seok Yun Kang, Sung Rok Kim, Hyun Joo Kim
PURPOSE: Neuropathic cancer pain (NCP) is a common and potentially debilitating symptom in cancer patients. We investigated the prevalence of NCP, as well as its management and association with QOL. METHODS: Cancer patients with pain ≥1 on the visual analogue scale (VAS) were surveyed with the Douleur Neuropathique (DN4) questionnaire, the Brief Pain Inventory-Short Form (BPI-SF), and the EuroQOL five dimensions (EQ-5D) questionnaire. The associations between NCP and pain severity or NCP and QOL, while controlling for variables relevant to QOL, were then analyzed...
July 8, 2017: Supportive Care in Cancer: Official Journal of the Multinational Association of Supportive Care in Cancer
https://www.readbyqxmd.com/read/28686885/a-radioactive-material-monitoring-system-using-multiple-gamma-spectroscopy-detectors-and-centroid-method
#15
Hankyeol Song, Dong-Hoon Lee, Seung-Jae Lee, Chaeyeong Lee, Chanwoo Park, Hyun-Il Kim, Jihoon Kang, Yong Hyun Chung
A radioactive material monitoring system, employing a passive detection technique with multiple gamma spectroscopy detectors and the centroid method for use in large areas, is presented. The system determines the location and the activity of radioisotopes. The proposed system was designed and evaluated using Monte Carlo simulations and experiments. In both simulation and experiment, calculated source locations were well distinguished and the location was determined within less than 1m range compared to the actual location...
June 26, 2017: Applied Radiation and Isotopes
https://www.readbyqxmd.com/read/28684329/house-dust-mite-increases-pro-th2-cytokines-il-25-and-il-33-via-the-activation-of-tlr1-6-signaling
#16
Yong Hyun Jang, Jin Kyeong Choi, Meiling Jin, Young-Ae Choi, Zae Young Ryoo, Hyun-Shik Lee, Pil-Hoon Park, Sun-Uk Kim, Taeg Kyu Kwon, Myoung Ho Jang, Sin-Hyeog Im, Sun Young Moon, Weon Ju Lee, Seok-Jong Lee, Do Won Kim, Sang-Hyun Kim
House dust mite (HDM) has been implicated in the etiology and exacerbation of atopic dermatitis (AD). Diverse factors contribute to HDM allergenicity through the activation of innate immunity. We investigated whether Dermatophagoides farinae extract (DFE) allergens mediate innate immune activation through specific Toll-like receptors (TLRs) in epidermal keratinocytes, DFE-induced murine AD model and human AD lesions. DFE activated the expression of TLR1, TLR6, interleukin (IL)-25, and IL-33 in human primary keratinocytes and HaCaT cells...
July 3, 2017: Journal of Investigative Dermatology
https://www.readbyqxmd.com/read/28683367/efficient-removal-of-arsenic-by-strategically-designed-and-layer-by-layer-assembled-ps-rgo-go-fe3o4-composites
#17
Bong Kyun Kang, Byeong Seok Lim, Yeojoon Yoon, Sung Hoon Kwag, Won Kyu Park, Young Hyun Song, Woo Seok Yang, Yong-Tae Ahn, Joon-Wun Kang, Dae Ho Yoon
The PS@+rGO@GO@Fe3O4 (PG-Fe3O4) hybrid composites for Arsenic removal were successfully fabricated and well dispersed using layer-by-layer assembly and a hydrothermal method. The PG-Fe3O4 hybrid composites were composed of uniformly coated Fe3O4 nanoparticles on graphene oxide layers with water flow space between 3D structures providing many contact area and adsorption sites for Arsenic adsorption. The PG-Fe3O4 hybrid composite has large surface adsorption sites and exhibits high adsorption capacities of 104 mg/g for As (III) and 68 mg/g for As (V) at 25 °C and pH 7 comparison with pure Fe3O4 and P-Fe3O4 samples...
July 3, 2017: Journal of Environmental Management
https://www.readbyqxmd.com/read/28682407/selective-protein-transport-through-ultra-thin-suspended-reduced-graphene-oxide-nanopores
#18
Dae-Sik Lee, Seokhan Park, Yong Duk Han, Jae Eun Lee, Hu Young Jeong, Hyun C Yoon, Moon Yeon Jung, Sang Ouk Kim, Sung-Yool Choi
The nanoporous free-standing graphene membrane is of great interest in high performance separation technology. In particular, the separation of biological molecules with similar sizes is one of the key challenges in the purification of biomaterials. Here, we report a reliable, cost-effective, and facile method for the fabrication of a graphene-based nanosieve and its application in the separation of similar-size proteins. A suspended reduced graphene oxide (rGO) nanosieve with ultra-thin, large-area, well-ordered, and dense 15 nm-sized pores was fabricated using block copolymer (BCP) lithography...
July 6, 2017: Nanoscale
https://www.readbyqxmd.com/read/28677356/age-based-causes-and-clinical-characteristics-of-immediate-type-food-allergy-in-korean-children
#19
Kyunguk Jeong, Jihyun Kim, Kangmo Ahn, So Yeon Lee, Taek Ki Min, Bok Yang Pyun, Yoon Hee Kim, Kyung Won Kim, Myung Hyun Sohn, Kyu Earn Kim, Gwang Cheon Jang, Tae Won Song, Jung Hee Kim, You Hoon Jeon, Yong Ju Lee, Yong Mean Park, Hye Yung Yum, Hyun Hee Kim, Woo Kyung Kim, Sooyoung Lee
PURPOSE: Age-based causes and clinical characteristics of immediate-type food allergy (FA) have not been sufficiently studied. Therefore, we investigated age-dependent clinical profiles of FA in Korean children through an extensive multicenter investigation. METHODS: Using a case report form developed by the authors, a retrospective medical record review was performed of patients (0-18 years old) diagnosed with immediate-type FA between September 2014 and August 2015 in 14 tertiary hospitals in Korea...
September 2017: Allergy, Asthma & Immunology Research
https://www.readbyqxmd.com/read/28677355/monoclonal-antibodies-to-recombinant-fag-e-3-buckwheat-allergen-and-development-of-a-two-site-elisa-for-its-quantification
#20
Kyoung Yong Jeong, Kyung Hee Park, Jae Hyun Lee, Jung Won Park
PURPOSE: Buckwheat is a major cause of anaphylaxis, and Fag e 3 is the key major allergen in buckwheat. However, an immunoassay system for the quantification of Fag e 3 has yet to be developed. METHODS: We developed a 2-site enzyme-linked immunosorbent assay (ELISA) using monoclonal antibodies (mAbs) produced against recombinant Fag e 3. We applied this ELISA to quantify native Fag e 3 in total buckwheat extract. RESULTS: Four clones of mAbs were produced, and all recognized vicilin allergens not only from buckwheat, but also from peanut and walnut...
September 2017: Allergy, Asthma & Immunology Research
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