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https://www.readbyqxmd.com/read/28090037/expression-of-pro-renin-receptor-during-rapamycin-induced-erythropoiesis-in-k562-erythroleukemia-cells-and-its-possible-dual-actions-on-erythropoiesis
#1
Kiriko Kaneko, Koji Ohba, Takuo Hirose, Kazuhito Totsune, Kazumichi Furuyama, Kazuhiro Takahashi
(Pro)renin receptor ((P)RR), a specific receptor for renin and prorenin, is expressed in erythroblastic cells. (P)RR has multiple biological actions: prorenin activation, stimulation of the intracellular signaling including extracellular signal-regulated kinases, and functional complex formation with vacuolar H(+)-ATPase (v-ATPase). However, the functional implication of (P)RR in erythroblast cells has not been clarified. The aim of the present study was to clarify changes of (P)RR expression during erythropoiesis and a role of (P)RR in the heme synthesis...
2017: Tohoku Journal of Experimental Medicine
https://www.readbyqxmd.com/read/28081156/generation-of-a-stable-transgenic-swine-model-expressing-a-porcine-histone-2b-egfp-fusion-protein-for-cell-tracking-and-chromosome-dynamics-studies
#2
Renan B Sper, Sehwon Koh, Xia Zhang, Sean Simpson, Bruce Collins, Jeff Sommer, Robert M Petters, Ignacio Caballero, Jeff L Platt, Jorge A Piedrahita
Transgenic pigs have become an attractive research model in the field of translational research, regenerative medicine, and stem cell therapy due to their anatomic, genetic and physiological similarities with humans. The development of fluorescent proteins as molecular tags has allowed investigators to track cell migration and engraftment levels after transplantation. Here we describe the development of two transgenic pig models via SCNT expressing a fusion protein composed of eGFP and porcine Histone 2B (pH2B)...
2017: PloS One
https://www.readbyqxmd.com/read/28078438/biological-monitoring-of-cobalt-in-hard-metal-factory-workers
#3
Andrea Princivalle, Ivo Iavicoli, Marzia Cerpelloni, Antonia Franceschi, Maurizio Manno, Luigi Perbellini
PURPOSE: The main aim of this study was to investigate the cobalt (Co) concentrations in urine along 4 months and their relationship with Co concentrations in blood and haemoglobin (adducts) in 34 workers from a hard metal manufacturing plant where metallic Co and Co oxide were used. Furthermore, the excretion kinetics of Co was investigated and the half-lives of Co in blood, plasma and urine were calculated along 18 days of non-exposure in the same workers. METHODS: Co was analysed, in all biological samples, by ICP/MS...
January 11, 2017: International Archives of Occupational and Environmental Health
https://www.readbyqxmd.com/read/28064319/-main-regulatory-element-mre-of-the-danio-rerio-%C3%AE-%C3%AE-globin-gene-domain-exerts-enhancer-activity-toward-the-promoters-of-the-embryonic-larval-and-adult-globin-genes
#4
A P Kovina, N V Petrova, S V Razin, O V Yarovaia
In warm-blooded vertebrates, the α- and β-globin genes are organized in domains of different types and are regulated in different fashion. In cold-blooded vertebrates and, in particular, the tropical fish Danio rerio, the α- and β-globin genes form two gene clusters. A major D. rerio globin gene cluster is in chromosome 3 and includes the α- and β-globin genes of embryonic-larval and adult types. The region upstream of the cluster contains c16orf35, harbors the main regulatory element (MRE) of the α-globin gene domain in warm-blooded vertebrates...
November 2016: Molekuliarnaia Biologiia
https://www.readbyqxmd.com/read/28060419/a-molecular-roadmap-of-definitive-erythropoiesis-from-human-induced-pluripotent-stem-cells
#5
Muhammad A Razaq, Stephen Taylor, David J Roberts, Lee Carpenter
Human induced pluripotent stem cells (hiPSCs) are being considered for use in understanding haematopoietic disorders and as a potential source of in vitro manufactured red cells. Here, we show that hiPSCs are able to recapitulate various stages of developmental erythropoiesis. We show that primitive erythroblasts arise first, express CD31(+) with CD235a(+) , embryonic globins and red cell markers, but fail to express the hallmark red cell transcripts of adult erythropoiesis. When hiPSC-derived CD45(+) CD235a(-) haematopoietic progenitors are isolated on day 12 and further differentiated on OP9 stroma, they selectively express CD36(+) and CD235a(+) , adult erythroid transcripts for transcription factors (e...
January 6, 2017: British Journal of Haematology
https://www.readbyqxmd.com/read/28060121/the-sickle-%C3%AE-thalassemia-phenotype
#6
Adekunle D Adekile, Nagihan Akbulut, Asmaa F Azab, Sundus Al-Sharida, Diana Thomas
Sβ-thalassemia (Sβ-thal) is common among Gulf Arab patients with sickle cell disease, but the phenotype of this group had not been well-documented. We have studied a group of Kuwaiti patients and compared the phenotype in the homozygotes (SS) and Sβ-thal patients. Complete blood count, hemoglobin quantitation, serum bilirubin, and lactate dehydrogenase were determined with standard techniques. The patients were screened for α-globin genotype. The Sβ-thal patients were also screened for the HBG2 Xmn-1 polymorphism...
January 5, 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/28053695/study-on-hydroxyurea-response-in-hemoglobinopathies-patients-using-genetic-markers-and-liquid-erythroid-cultures
#7
Serena Sclafani, Alice Pecoraro, Veronica Agrigento, Antonio Troia, Rosario Di Maggio, Massimiliano Sacco, Aurelio Maggio, Elena D'Alcamo, Rosalba Di Marzo
Increased expression of fetal hemoglobin (HbF) may ameliorate the clinical course of hemoglobinopathies. Hydroxyurea (HU) is the only inducer approved for the treatment of these diseases able to stimulate HbF production but patients' response is highly variable indicating the utility of the identification of pharmacogenomic biomarkers in order to predict pharmacological treatment efficacy. To date few studies to evaluate the role of genetic determinants in HU response have been conducted showing contradictory results...
November 2, 2016: Hematology Reports
https://www.readbyqxmd.com/read/28032989/how-does-your-protein-fold-elucidating-the-apomyoglobin-folding-pathway
#8
H Jane Dyson, Peter E Wright
Although each type of protein fold and in some cases individual proteins within a fold classification can have very different mechanisms of folding, the underlying biophysical and biochemical principles that operate to cause a linear polypeptide chain to fold into a globular structure must be the same. In an aqueous solution, the protein takes up the thermodynamically most stable structure, but the pathway along which the polypeptide proceeds in order to reach that structure is a function of the amino acid sequence, which must be the final determining factor, not only in shaping the final folded structure, but in dictating the folding pathway...
December 29, 2016: Accounts of Chemical Research
https://www.readbyqxmd.com/read/28031553/genetic-origin-of-beh%C3%A3-et-s-disease-population-in-denizli-turkey-population-genetics-data-analysis-historical-demography-and-geographical-perspectives-based-on-%C3%AE-globin-gene-cluster-haplotype-variation
#9
O Ozturk, S Arikan, A Bahadir, A Atalay, E O Atalay
In our study, we aimed to investigate the possible genetic drift, relationships, expansion and historical origin based on haplotype frequencies of the β-globin gene cluster of normal and Behçet's disease (BD) population in Denizli, Turkey. We examined blood DNA samples obtained from our DNA bank. The association of population genetic parameters such as haplotypes, diversity, differentiation, Hardy-Weinberg equilibrium and demographic analysis for two populations was performed by Arlequin ver. 3.5. Our results show that both populations have high similarity in genetic parameters in terms of development and expansion based on haplotype diversity through the history...
December 29, 2016: Genes and Immunity
https://www.readbyqxmd.com/read/28026072/activation-of-klf1-enhances-the-differentiation-and-maturation-of-red-blood-cells-from-human-pluripotent-stem-cells
#10
Cheng-Tao Yang, Rui Ma, Richard A Axton, Melany Jackson, A Helen Taylor, Antonella Fidanza, Lamin Marenah, Jan Frayne, Joanne C Mountford, Lesley M Forrester
Blood transfusion is widely used in the clinic but the source of red blood cells (RBCs) is dependent on donors, procedures are susceptible to transfusion-transmitted infections and complications can arise from immunological incompatibility. Clinically-compatible and scalable protocols that allow the production of RBCs from human embryonic stem cells (hESCs) and induced pluripotent stem cells (iPSCs) have been described but progress to translation has been hampered by poor maturation and fragility of the resultant cells...
December 27, 2016: Stem Cells
https://www.readbyqxmd.com/read/28007975/ctdgfinder-a-novel-homology-based-algorithm-for-identifying-closely-spaced-clusters-of-tandemly-duplicated-genes
#11
Juan F Ortiz, Antonis Rokas
Closely spaced clusters of tandemly duplicated genes (CTDGs) contribute to the diversity of many phenotypes, including chemosensation, snake venom, and animal body plans. CTDGs have traditionally been identified subjectively as genomic neighborhoods containing several gene duplicates in close proximity; however, CTDGs are often highly variable with respect to gene number, intergenic distance, and synteny. This lack of formal definition hampers the study of CTDG evolutionary dynamics and the discovery of novel CTDGs in the exponentially growing body of genomic data...
October 20, 2016: Molecular Biology and Evolution
https://www.readbyqxmd.com/read/28007617/a-novel-tandem-mass-spectrometry-method-for-first-line-screening-of-mainly-beta-thalassemia-from-dried-blood-spots
#12
Chaowen Yu, Shuodan Huang, Ming Wang, Juan Zhang, Hao Liu, Zhaojian Yuan, Xingbin Wang, Xiaoyan He, Jie Wang, Lin Zou
: Traditional methods for thalassemia screening are time-consuming and easily affected by cell hemolysis or hemoglobin degradation in stored blood samples. Tandem mass spectrometry (MS/MS) proved to be an effective technology for sickle cell disorders (SCD) screening. Here, we developed a novel MS/MS method for β-thalassemia screening from dried blood spots (DBS). Stable isotopic-labeled peptides were used as internal standards for quantification and calculation of the α:β-globin ratios...
December 20, 2016: Journal of Proteomics
https://www.readbyqxmd.com/read/28007020/hb-a2-episkopi-a-novel-%C3%AE-globin-chain-variant-hbd-c-428c-t-in-a-family-of-mixed-cypriot-lebanese-descent
#13
Carsten W Lederer, Eleni Pavlou, George A Tanteles, Paola Evangelidou, Carolina Sismani, Annita Kolnagou, Maria Sitarou, Soteroulla Christou, Michael Hadjigavriel, Marina Kleanthous
OBJECTIVES: Thalassaemia is a potentially lethal inherited anaemia, caused by reduced or absent synthesis of globin chains. Measurement of the minor adult haemoglobin Hb A2, combining α- with δ-globin, is critical for the routine diagnosis of carrier status for α- or β-thalassaemia. Here, we aim to characterize a novel δ-globin variant, Hb A2 Episkopi, in a single family of mixed Lebanese and Cypriot ancestry with mild hypochromic anaemia and otherwise normal globin genotype, which also presents with a coincidental 0...
December 22, 2016: Hematology (Amsterdam, Netherlands)
https://www.readbyqxmd.com/read/28003571/identification-of-%C3%AE-globin-haplotypes-linked-to-sickle-hemoglobin-hb-s-alleles-in-mazandaran-province-iran
#14
Faeghe Aghajani, Mohammad Reza Mahdavi, Mehrnoush Kosaryan, Mehrad Mahdavi, Mohaddese Hamidi, Hossein Jalali
Carrier frequency of the β(S) allele has been reported to be 0.19% in Mazandaran province, northern Iran. Haplotype analysis of the β(S) allele helps trace the origin of its encoded hemoglobin (Hb) variant, Hb S, in a region. The aim of this study was to investigate the haplotypes associated with β(S) alleles in Mazandaran province. Capillary electrophoresis was carried out to detect individuals suspected to have a βS allele(s). DNA analysis (PCR-RFLP) was used for final confirmation. To identify 5\' to 3\' β-globin gene cluster haplotypes associated with β(S) alleles, family linkage analysis was applied...
December 21, 2016: Genes & Genetic Systems
https://www.readbyqxmd.com/read/27995414/na-h-exchanger-3-inhibitor-diminishes-hepcidin-enhanced-duodenal-calcium-transport-in-hemizygous-%C3%AE-globin-knockout-thalassemic-mice
#15
Narattaphol Charoenphandhu, Kamonshanok Kraidith, Kornkamon Lertsuwan, Chanakarn Sripong, Panan Suntornsaratoon, Saovaros Svasti, Nateetip Krishnamra, Kannikar Wongdee
Recent investigation has shown that the liver-derived iron-regulating hormone, hepcidin, can potentiate intestinal calcium absorption in hemizygous β-globin knockout thalassemic (BKO) mice. Since the upregulation of Fe(2+) and H(+) cotransporter, divalent metal transporter (DMT)-1, has been shown to correlate with thalassemia-induced intestinal calcium absorption impairment, the inhibition of the apical Na(+)/H(+) exchanger (NHE)-3 that is essential for cytoplasmic pH regulation and transepithelial sodium absorption was hypothesized to negatively affect hepcidin action...
December 19, 2016: Molecular and Cellular Biochemistry
https://www.readbyqxmd.com/read/27989134/stability-and-application-of-reactive-nitrogen-and-oxygen-species-induced-hemoglobin-modifications-in-dry-blood-spots-as-analyzed-by-liquid-chromatography-tandem-mass-spectrometry
#16
Hauh-Jyun Candy Chen, Chih-Huang Fan, Ya-Fen Yang
Dried blood spot (DBS) is an emerging microsampling technique for the bioanalysis of small molecules, including fatty acids, metabolites, drugs, and toxicants. DBS offers many advantages as a sample format including easy sample collection and cheap sample shipment. Hemoglobin adducts have been recognized as a suitable biomarker for monitoring chemical exposure. We previously reported that certain modified peptides in hemoglobin derived from reactive chlorine, nitrogen, and oxygen species are associated with factors including smoking, diabetes mellitus, and aging...
December 19, 2016: Chemical Research in Toxicology
https://www.readbyqxmd.com/read/27987932/by-products-of-scyliorhinus-canicula-prionace-glauca-and-raja-clavata-a-valuable-source-of-predominantly-6s-sulfated-chondroitin-sulfate
#17
Ramon Novoa-Carballal, Ricardo Pérez-Martín, María Blanco, Carmen G Sotelo, Dario Fassini, Cláudia Nunes, Manuel A Coimbra, Tiago H Silva, Rui L Reis, José Antonio Vázquez
Chondroitin sulfate (CS) was isolated from Scyliorhinus canicula (fin, head and skeleton), Prionace glauca (head), and Raja clavata (skeleton) by-products from fish processing industry using environmentally friendly processes. The molecular weight was determined by gel permeation chromatography and the sugar composition and sulfation position by NMR and SAX-HPLC after enzymatic digestion. The CSs showed a prevalent 6S GalNAc sulfation for the 3 species (4S/6S ratio lower than 1). A higher 6S sulfation was observed for P...
February 10, 2017: Carbohydrate Polymers
https://www.readbyqxmd.com/read/27987695/self-fluorescent-drug-delivery-vector-based-on-genipin-crosslinked-polyethylenimine-conjugated-globin-nanoparticle
#18
Yan Zhang, Lina Mao, Juan Liu, Tianjun Liu
A kind of self-fluorescent, biocompatible, and low-toxic Genipin crosslinked Globin-PEI nanoparticle (Gb-G-PEI NP) with high enzymolysis-stability and photo-stability was synthesized successfully. The properties of the Gb-G-PEI NP were characterized, including its particle size, surface zeta potential, morphology, paclitaxel (PTX) loading capacity and release. The Gb-G-PEI NPs as imaging probe were investigated by Confocal Laser Scanning Microscope (CLSM) in vitro and by fluorescence imaging system in vivo...
February 1, 2017: Materials Science & Engineering. C, Materials for Biological Applications
https://www.readbyqxmd.com/read/27981643/ugt1a1-ta-n-genotype-is-not-the-major-risk-factor-of-cholelithiasis-in-sickle-cell-disease-children
#19
Philippe Joly, Céline Renoux, Philippe Lacan, Yves Bertrand, Giovanna Cannas, Nathalie Garnier, Daniella Cuzzubbo, Kamila Kebaïli, Cécile Renard, Alexandra Gauthier, Vincent Pialoux, Cyril Martin, Marc Romana, Philippe Connes
Because of the increased hemolytic rate, a significant proportion of patients with sickle cell disease (SCD) are prone to develop cholelithiasis. The present study investigated the role of several genetic factors (UGT1A1 promoter (TA)n repeat polymorphism, alpha-globin status), hematological parameters, clinical severity and hydroxyurea (HU) therapy on the occurrence of cholelithiasis in SCD METHODS: One hundred and fifty eight children (2-18 years old) and regularly followed at the university hospital of Lyon (France) were included...
December 16, 2016: European Journal of Haematology
https://www.readbyqxmd.com/read/27979672/-neonatal-expression-of-beta-thalassemia-trait-associated-with-hereditary-spherocytosis-in-two-monozygotic-twins
#20
H Ben Hamouda, B Mahjoub, H Soua, S Laradi, A Miled, M T Sfar
The beta-thalassemia trait is a heterozygous beta-thalassemia characterized by a partial deficiency of the synthesis of beta-globin chains of hemoglobin. It is usually asymptomatic and the diagnosis is often made on the occasion of the completion of a systematic blood count or a family survey. Clinical expression during the neonatal period is impossible and its association with hereditary spherocytosis is common. We report two monozygotic twins who, on the 3rd day of life, presented intense jaundice, unconjugated bilirubin associated with anemia, without hepatosplenomegaly...
December 12, 2016: Archives de Pédiatrie: Organe Officiel de la Sociéte Française de Pédiatrie
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