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https://www.readbyqxmd.com/read/28924445/the-first-argentinian-family-with-familial-amyloidosis-of-the-finnish-type
#1
Francisco Lucero Saá, Federico Andrés Cremona, Natalia Ximena Mínguez, María Laura Igarzabal, Pablo Chiaradía
Familial amyloidosis of the Finnish type or Meretoja syndrome is a rare autosomic dominant inherited systemic condition. It was first described by Meretoja in Finland in 1969. It is a disease produced by a single mutation in the gene coding for gelsolin, which generates an abnormal protein that cumulates in tissues and leads to various signs. Obtaining an early diagnosis can be challenging, as the first manifestations of the disease are ophthalmological and may only be seen with slit-lamp biomicroscopy. We present the first 3 cases diagnosed in Argentina, confirmed by genetic molecular testing...
May 2017: Case Reports in Ophthalmology
https://www.readbyqxmd.com/read/28881733/positive-regulation-of-taz-expression-by-ebv-lmp1-contributes-to-cell-proliferation-and-epithelial-mesenchymal-transition-in-nasopharyngeal-carcinoma
#2
Jiang He, Feiyu Tang, Liyu Liu, Lin Chen, Jiang Li, Danming Ou, Lu Zhang, Zhi Li, Deyun Feng, Wenzheng Li, Lun-Quan Sun
The Epstein-Barr virus latent membrane protein 1 (LMP1) is an integral membrane protein. LMP1 has been reported to activate the NF-κB and mitogen-activated protein kinase pathways. However, these effects alone are unable to account for the profound oncogenic properties of LMP1. TAZ is one of the nuclear effectors of Hippo-related pathways and highly expressed in many human tumors. Here, we reported that TAZ was frequently expressed in LMP1-positive nasopharyngeal carcinoma. In NPC cell lines, we showed that LMP1 promoted TAZ expression...
August 8, 2017: Oncotarget
https://www.readbyqxmd.com/read/28872708/validation-of-an-automated-immune-turbidimetric-assay-for-serum-gelsolin-and-its-possible-clinical-utility-in-sepsis
#3
Zoltán Horváth-Szalai, Péter Kustán, Balázs Szirmay, Ágnes Lakatos, Per H Christensen, Tamás Huber, Beáta Bugyi, Diána Mühl, Andrea Ludány, Attila Miseta, Gábor L Kovács, Tamás Kőszegi
BACKGROUND: Studies showing the potential predictive value of the actin-binding protein gelsolin, in critically ill patients are scarce. Moreover, even up to now a rapid automated measurement of gelsolin has still remained a challenge. Therefore, we developed and validated an automated serum gelsolin immune turbidimetric assay for possible clinical use. METHODS: Validation of serum gelsolin assay was performed on a Cobas 8000/c502 analyzer (Roche) according to the second edition of Eurachem guidelines...
September 5, 2017: Journal of Clinical Laboratory Analysis
https://www.readbyqxmd.com/read/28835422/tumor-associated-macrophages-promote-epigenetic-silencing-of-gelsolin-through-dna-methyltransferase-1-in-gastric-cancer-cells
#4
Hao-Chen Wang, Chin-Wang Chen, Chia-Lung Yang, I-Min Tsai, Ya-Chin Hou, Chang-Jung Chen, Yan-Shen Shan
Epigenetic repression of the tumor suppressor gelsolin (GSN) is frequently observed in cancers. Chronic inflammation can promote tumor progression via aberrant DNA methylation. In this study, we investigated the role of tumor-associated macrophages (TAMs) in DNA methylation of the GSN gene during gastric cancer (GC) progression. Immunofluorescence staining of 121 GC tissues showed aberrant localization of GSN and DNA methyltransferase 1 (DNMT1) and juxtaposition of DNMT1 and M2 TAMs. Decreased GSN protein and mRNA expression and increased DNA methylation in the GSN promoter were observed in GC cell lines and clinical specimens...
August 23, 2017: Cancer Immunology Research
https://www.readbyqxmd.com/read/28835069/-changes-of-content-and-mrna-expression-of-gelsolin-and-proliferation-activity-of-t-lymphocyte-in-spleen-of-mice-with-severe-burn-injury
#5
Q Chen, H M Yang
Objective: To investigate the changes of content and mRNA expression of gelsolin and proliferation activity of T-lymphocyte in spleen of mice with severe burn injury, so as to determine the optimum intervention time of gelsolin. Methods: Eighty male BALB/c mice were divided into sham injury group and burn group according to the random number table, with 40 mice in each group. Mice in burn group were inflicted with 15% total body surface area full-thickness scald (hereinafter referred to as burn) on the back...
August 20, 2017: Zhonghua Shao Shang za Zhi, Zhonghua Shaoshang Zazhi, Chinese Journal of Burns
https://www.readbyqxmd.com/read/28828707/pathology-and-diagnosis-of-renal-non-al-amyloidosis
#6
REVIEW
Sanjeev Sethi, Jason D Theis
Renal amyloidosis is characterized by acellular Congo red positive deposits in the glomeruli, interstitium and/or arteries. Light chain restriction on immunofluorescence studies is present in AL-amyloidosis, the most common type of amyloidosis involving the kidney. The detection of Congo red positive deposits coupled with negative immunofluorescence studies is highly suggestive of non-AL amyloidosis. Some of the non-AL amyloidosis are common while others are relatively rare. The clinical features, laboratory and renal pathology findings are helpful in the diagnosis and typing of non-AL amyloidosis...
August 21, 2017: Journal of Nephrology
https://www.readbyqxmd.com/read/28814713/gelsolin-dysfunction-causes-photoreceptor-loss-in-induced-pluripotent-cell-and-animal-retinitis-pigmentosa-models
#7
Roly Megaw, Hashem Abu-Arafeh, Melissa Jungnickel, Carla Mellough, Christine Gurniak, Walter Witke, Wei Zhang, Hemant Khanna, Pleasantine Mill, Baljean Dhillon, Alan F Wright, Majlinda Lako, Charles Ffrench-Constant
Mutations in the Retinitis Pigmentosa GTPase Regulator (RPGR) cause X-linked RP (XLRP), an untreatable, inherited retinal dystrophy that leads to premature blindness. RPGR localises to the photoreceptor connecting cilium where its function remains unknown. Here we show, using murine and human induced pluripotent stem cell models, that RPGR interacts with and activates the actin-severing protein gelsolin, and that gelsolin regulates actin disassembly in the connecting cilium, thus facilitating rhodopsin transport to photoreceptor outer segments...
August 16, 2017: Nature Communications
https://www.readbyqxmd.com/read/28794177/discovery-and-validation-of-a-six-marker-serum-protein-signature-for-the-diagnosis-of-active-pulmonary-tuberculosis
#8
Mary A De Groote, David G Sterling, Thomas Hraha, Theresa Russell, Louis S Green, Kirsten Wall, Stephan Kraemer, Rachel Ostroff, Nebojsa Janjic, Urs A Ochsner
New non-sputum biomarker tests for active tuberculosis (TB) diagnostics are of highest priority for global TB control. We performed in-depth proteomic analysis using the 4000-plex SOMAscan assay on 1470 serum samples from seven TB-endemic countries. All samples were from patients with symptoms and signs suggestive of active pulmonary TB that were systematically confirmed or ruled-out for TB by culture and clinical follow-up. HIV-coinfection was present in 34%and 22% were sputum smear-negative. Serum protein biomarkers were identified by stability selection using L1-regularized logistic regression and by Kolmogorov-Smirnov (KS) statistics...
August 9, 2017: Journal of Clinical Microbiology
https://www.readbyqxmd.com/read/28791367/molecular-mechanisms-of-breast-cancer-metastasis-by-gene-expression-profile-analysis
#9
Tianying Zheng, Aijun Wang, Dongyan Hu, Yonggang Wang
Metastasis is the main cause of breast cancer‑related mortalities. The present study aimed to uncover the relevant molecular mechanisms of breast cancer metastasis and to explore potential biomarkers that may be used for prognosis. Expression profile microarray data GSE8977, which contained 22 stroma samples (15 were from normal breast and 7 were from invasive ductal carcinoma tumor samples), were obtained from the Gene Expression Omnibus database. Following data preprocessing, differentially expressed genes (DEGs) were selected based on analyses conducted using the linear models for microarray analysis package from R and Bioconductor software...
October 2017: Molecular Medicine Reports
https://www.readbyqxmd.com/read/28757820/nonconventional-markers-of-sepsis
#10
Péter Kustán, Zoltán Horváth-Szalai, Diána Mühl
Sepsis still remains a challenging healthcare problem with high mortality rate. To improve outcome, early diagnosis and monitoring of sepsis is of utmost importance. In this process objective laboratory parameters are the most helpful. Procalcitonin and C-reactive protein are the most commonly used and recommended markers of sepsis however, more than 200 sepsis biomarkers have already been published. This mini review focuses on nonconventional novel possibilities for the recognition of sepsis severity. Presepsin, actin and actin scavenger proteins (gelsolin and Gc-globulin) and orosomucoid are discussed...
May 2017: EJIFCC
https://www.readbyqxmd.com/read/28755273/cognitive-impairment-and-brain-and-peripheral-alterations-in-a-murine-model-of-intraventricular-hemorrhage-in-the-preterm-newborn
#11
Antonio Segado-Arenas, Carmen Infante-Garcia, Isabel Benavente-Fernandez, Daniel Sanchez-Sotano, Juan Jose Ramos-Rodriguez, Almudena Alonso-Ojembarrena, Simon Lubian-Lopez, Monica Garcia-Alloza
Germinal matrix hemorrhage-intraventricular hemorrhage (GMH-IVH) remains a serious complication in the preterm newborn. The significant increase of survival rates in extremelye preterm newborns has also contributed to increase the absolute number of patients developing GMH-IVH. However, there are relatively few available animal models to understand the underlying mechanisms and peripheral markers or prognostic tools. In order to further characterize central complications and evolution of GMH-IVH, we injected collagenase intraventricularly to P7 CD1 mice and assessed them in the short (P14) and the long term (P70)...
July 28, 2017: Molecular Neurobiology
https://www.readbyqxmd.com/read/28700355/identification-of-cd14-as-a-potential-biomarker-of-hepatocellular-carcinoma-using-itraq-quantitative-proteomics
#12
Jiao Guo, Rui Jing, Jian-Hong Zhong, Xin Dong, Yun-Xi Li, Yin-Kun Liu, Tian-Ren Huang, Chun-Yan Zhang
Hepatocellular carcinoma (HCC) is one of the most common malignant tumors without effective diagnostic biomarkers. This study intended to dynamically analyze serum proteomics in different pathological stages of liver diseases, and discover potential diagnostic biomarkers for early HCC. Patients with hepatitis B virus (HBV) infection, liver cirrhosis (LC), or HCC together with healthy controls (HC) were enrolled. Proteins differentially expressed between groups were screened using isobaric tagging for relative and absolute quantitation (iTRAQ), and promising HCC biomarker candidates were subjected to bioinformatics analysis, including K-means clustering, gene ontology (GO) and string network analysis...
June 28, 2017: Oncotarget
https://www.readbyqxmd.com/read/28680082/visualizing-temperature-mediated-activation-of-gelsolin-and-its-deactivation-by-pip2-a-saxs-based-study
#13
Maulik D Badmalia, Shikha Singh, Renu Garg, Ashish
This is the first report describing temperature based initiation of gelsolin's F-actin depolymerization activity, even in absence of free Ca(2+) or low pH. Small angle X-ray scattering (SAXS) and circular dichroism (CD) studies revealed that temperature in the range of 30-40 °C is capable of opening the G1 domain alone, as remaining domains are held together by the Ca(2+)-sensitive C-tail latch without any loss in the secondary structural content. Full opening of all domains of tail-less gelsolin, and retention of closed shape for G2-G6 gelsolin merely by heating, further substantiated our findings...
July 5, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28673451/a-prospective-proteomics-study-identified-potential-biomarkers-of-encapsulating-peritoneal-sclerosis-in-peritoneal-effluent
#14
Vasileios Zavvos, Anthony T Buxton, Caroline Evans, Mark Lambie, Simon J Davies, Nicholas Topley, Martin Wilkie, Angela Summers, Paul Brenchley, Dimitrios S Goumenos, Timothy S Johnson
Encapsulating peritoneal sclerosis (EPS) is a potentially devastating complication of peritoneal dialysis (PD). Diagnosis is often delayed due to the lack of effective and accurate diagnostic tools. We therefore examined peritoneal effluent for potential biomarkers that could predict or confirm the diagnosis of EPS and would be valuable in stratifying at-risk patients and driving appropriate interventions. Using prospectively collected samples from the Global Fluid Study and a cohort of Greek PD patients, we utilized 2D SDSPAGE/ MS and iTRAQ to identify changes in the peritoneal effluent proteome from patients diagnosed with EPS and controls matched for treatment exposure...
July 1, 2017: Kidney International
https://www.readbyqxmd.com/read/28647184/identification-of-novel-biomarkers-for-adult-onset-immunodeficiency-aoid-syndrome-using-serum-proteomics
#15
Jeerang Wongtrakul, Thananya Thongtan, Sittiruk Roytrakul, Jutarat Praparattanapan, Jiraprapa Wipasa, Benjawan Kumrapich, Khuanchai Supparatpinyo
OBJECTIVE: To identify the candidate protein biomarkers of adult-onset-immunodeficiency (AOID) syndrome using serum proteomics. METHODS: Screening and verification phases were performed in the study. A total of 97 serum samples were classified into three groups: AOID patients with opportunistic infections (active AOID), AOID patients without opportunistic infections (inactive AOID), and healthy control. In the screening phase, pooled sera collected from patients and healthy control in each group were separated by 2D-gel electrophoresis, analyzed for differentially expressed proteins and identified for biomarkers using LC/MS...
May 2017: Asian Pacific Journal of Tropical Medicine
https://www.readbyqxmd.com/read/28640320/molecular-signature-of-coronary-stent-thrombosis-oxidative-stress-and-innate-immunity-cells
#16
Judit Cubedo, Ana Blasco, Teresa Padro, Ilaria Ramaiola, Oriol Juan-Babot, Javier Goicolea, José Antonio Fernández-Díaz, Juan Francisco Oteo, Lina Badimon
The clinical impact of in-stent thrombosis is high because it is associated with high mortality and 20 % of the patients suffer a recurrent event within the two following years. The aim of this study was to characterise the morphologic and proteomic profile of in-stent thrombi (IST) in comparison to thrombi developed on native coronary arteries (CT) to identify a differential molecular signature. The study included 45 patients with ST-elevation-myocardial-infarction (STEMI) treated by primary-percutaneous-intervention and thrombus aspiration: 21 had IST and 24 had CT...
August 30, 2017: Thrombosis and Haemostasis
https://www.readbyqxmd.com/read/28622474/larch-arabinogalactan-attenuates-myocardial-injury-by-inhibiting-apoptotic-cascades-in-a-rat-model-of-ischemia-reperfusion
#17
Sun-Ha Lim
We reported previously that supplementation with apple pectin, a dietary fiber, reduced myocardial injury in a rat model of ischemia-reperfusion. In this study, we further investigated an arabinogalactan, one of the constituent polysaccharides of pectin, to determine which domains comprising pectin were responsible for the protection. In a rat model of 30-min ischemia followed by 3-h reperfusion, supplementation with larch arabinogalactan (LAG) over 50 mg/kg/day significantly reduced infarct size. Reverse transcription polymerase chain reaction (RT-PCR), immunohistochemistry, and immunoblot analyses showed that intake of LAG blocked the steps involved in apoptotic cascades through downregulation of gelsolin gene expression at the protein (Gelsolin) level, inhibition of p38 phosphorylation in mitogen-activated protein kinase (MAPK) pathways, decreased bax/bcl-2 ratio at the protein (Bax/Bcl-2) level, which was correlated with the ratio at the mRNA level, inhibition of the conversion of Procaspase protein to Caspase-3 protein, and consequently a decrease in apoptotic cells...
July 2017: Journal of Medicinal Food
https://www.readbyqxmd.com/read/28605435/aav9-delivered-bispecific-nanobody-attenuates-amyloid-burden-in-the-gelsolin-amyloidosis-mouse-model
#18
Adriaan Verhelle, Nisha Nair, Inge Everaert, Wouter Van Overbeke, Lynn Supply, Olivier Zwaenepoel, Cindy Peleman, Jo Van Dorpe, Tony Lahoutte, Nick Devoogdt, Wim Derave, Marinee K Chuah, Thierry VandenDriessche, Jan Gettemans
No abstract text is available yet for this article.
August 1, 2017: Human Molecular Genetics
https://www.readbyqxmd.com/read/28599499/comparative-secretome-of-ovarian-serous-carcinoma-gelsolin-in-the-spotlight
#19
Sandra Pierredon, Pascale Ribaux, Jean-Christophe Tille, Patrick Petignat, Marie Cohen
Ovarian cancer is one of the most common types of reproductive cancer, and has the highest mortality rate amongst gynecological cancer subtypes. The majority of ovarian cancers are diagnosed at an advanced stage, resulting in a five-year survival rate of ~30%. Early diagnosis of ovarian cancer has improved the five-year survival rate to ≥90%, thus the current imperative requirement is to identify biomarkers that would allow the early detection, diagnosis and monitoring of the progression of the disease, or of novel targets for therapy...
June 2017: Oncology Letters
https://www.readbyqxmd.com/read/28582578/proteomics-of-the-epicardial-fat-secretome-and-its-role-in-post-operative-atrial-fibrillation
#20
Alessandro Viviano, Xiaoke Yin, Anna Zampetaki, Marika Fava, Mark Gallagher, Manuel Mayr, Marjan Jahangiri
Aims: Post-operative atrial fibrillation (POAF) is a predictor of morbidity and mortality after cardiac surgery. Latent predisposing factors may reside in the epicardial adipose tissue (EAT) due to its anatomical position and high protein production rate. In order to explore a possible mechanistic link, we characterized proteins secreted by the EAT preceding the onset of POAF. Methods and results: Epicardial adipose tissue samples were collected from 76 consecutive patients with no history of AF undergoing coronary artery bypass surgery, 50 samples for proteomic analysis and 26 for gene expression studies, further divided according to development of POAF...
June 5, 2017: Europace: European Pacing, Arrhythmias, and Cardiac Electrophysiology
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