Search results for "Globin"
showing 10 items of 734 documents
P68 A diet rich in wheat alpha-amylase/trypsin inhibitors (ATIs) enhances disease progression in the MRL-Fas(lpr) mouse model of systemic lupus eryth…
2020
Background Wheat alpha-amylase/trypsin inhibitors (ATIs) are the second most prevalent proteins in wheat (3–4% vs 80–90% for gluten) and potent activators of the innate immune system via the toll like receptor 4 (TLR4)-MD2-CD14 complex in cells of the mononuclear phagocyte system (Junker Y et al, J Exp Med 2012), triggering several autoimmune/inflammatory diseases. In contrast, pure gluten that is de-enriched of ATIs shows no stimulatory activity. MRL-Fas(lpr) mice develop progressive and spontaneous glomerular, tubulointerstitial and perivascular kidney disease, arthritis, lymphadenopathy, splenomegaly and circulating autoantibodies in a syndrome that resembles systemic lupus erythematosus…
Hemoglobin point-of-care testing: the HemoCue system.
2013
Besides the use of traditional laboratory resources, the diagnosis of anemia can also be accomplished by assessing hemoglobin (Hb) concentration with point-of-care testing (POCT) devices such as the HemoCue test systems. In several situations, these devices might suitably replace traditional laboratory testing, including several areas of health care where a very rapid Hb measurement might be required to make immediate therapeutic decisions. The use of these devices, however, should fulfill some basic criteria, including economic, clinical, and regulatory issues; appropriate training of the users and knowledge of test requirements, performance, limitations, and potential interferences; the u…
BCL11A intellectual developmental disorder: defining the clinical spectrum and genotype-phenotype correlations
2021
AbstractPurposeHeterozygous variants in BCL11A underlie an intellectual developmental disorder with persistence of fetal hemoglobin (BCL11A-IDD, a.k.a. Dias-Logan syndrome). We sought to delineate the genotypic and phenotypic spectrum of BCL11A-IDD.MethodsWe performed an in-depth analysis of 42 patients with BCL11A-IDD ascertained through a collaborative network of clinical and research colleagues. We also reviewed 33 additional affected individuals previously reported in the literature or available through public repositories with clinical information.ResultsMolecular and clinical data analysis of 75 patients with BCL11A-IDD identified 60 unique variants (30 frameshift, 7 missense, 6 splic…
Hemoglobin Level Analysis in Hemodialysis Patients Treated With Erythropoiesis Stimulating Agents
2010
In this chapter authors try to develop an expert system with the help of neural network method like Organizing Maps (SOMs) for hemodialysis patient.  Neural network models play a very important role for data analysis of hemodialysis patients with end-stage renal disease.  There are two main goals: firstly, the knowledge extraction from a database using Self-Organizing Maps (SOMs); and secondly, to provide an accurate prediction of Hb levels next month.
The role of pH on instability and aggregation of sickle hemoglobin solutions
2004
Understanding the physical basis of protein aggregation covers strong physical and biomedical interests. Sickle hemoglobin (HbS) is a point-mutant form of normal human adult hemoglobin (HbA). It is responsible for the first identified "molecular disease," as its propensity to aggregation is responsible for sickle cell disease. At moderately higher than physiological pH value, this propensity is inhibited: The rate of aggregate nucleation becomes exceedingly small and solubility after polymerization increases. These order-of-magnitude effects on polymer nucleation rates and concurrent relatively modest changes of solubility after polymerization are here shown to be related to both pH-induced…
Selective protein removal and desalting using microchip CE.
2006
Abstract This paper describes the on-line sample pretreatment and analysis of proteins and peptides with a poly(methylmethacrylate) (PMMA) microfluidic device (IonChip™). This chip consists of two hyphenated electrophoresis channels with integrated conductivity detectors. The first channel can be used for sample preconcentration and sample clean-up, while in the second channel the selected compounds are separated. Isotachophoresis (ITP) combined with zone electrophoresis (CZE) was used to preconcentrate a myoglobin sample by a factor of about 65 before injection into the second dimension and to desalt a mixture of six proteins with 100 mM NaCl. However, ITP–CZE could not be used for the rem…
Human neuroglobin: crystals and preliminary X-ray diffraction analysis
2002
Neuroglobin, a recently discovered member of the haemoglobin superfamily, is primarily expressed in the brain of humans and other vertebrates, where it has been proposed to enhance O(2) supply in response to hypoxia or ischaemia, protecting the neuron from hypoxic injury. Neuroglobin is the first example of a vertebrate haemoglobin in which a hexacoordinate haem geometry has been detected. A triple mutant (replacing three Cys residues) of human neuroglobin (151 amino acids) has been expressed in Escherichia coli, purified and crystallized in two crystal forms, the best of which diffracts to 1.95 A resolution using synchrotron radiation. The crystals belong to space group P2(1), with unit-ce…
The protein dynamical transition does not require the protein polypeptide chain
2011
We give experimental evidence that the main features of protein dynamics revealed by neutron scattering, i.e., the “protein dynamical transition” and the “boson peak”, do not need the protein polypeptide chain. We show that a rapid increase of hydrogen atoms fluctuations at about 220 K, analogous to the one observed in hydrated myoglobin powders, is also observed in a hydrated amino acids mixture with the chemical composition of myoglobin but lacking the polypeptide chain; in agreement with the protein behavior, the transition is abolished in the dry mixture. Further, an excess of low-frequency vibrational modes around 3 meV, typically observed in protein powders, is also observed in our mi…
Oxygen-induced changes in hemoglobin expression in Drosophila
2008
The hemoglobin gene 1 (dmeglob1) of the fruit fly Drosophila melanogaster is expressed in the tracheal system and fat body, and has been implicated in hypoxia resistance. Here we investigate the expression levels of dmeglob1 and lactate dehydrogenase (a positive control) in embryos, third instar larvae and adult flies under various regimes of hypoxia and hyperoxia. As expected, mRNA levels of lactate dehydrogenase increased under hypoxia. We show that expression levels of dmeglob1 are decreased under both short- and long-term hypoxia, compared with the normoxic (21% O2) control. By contrast, a hypoxia/reoxygenation regime applied to third instar larvae elevated the level of dmeglob1 mRNA. A…
Binding of water-soluble, globular proteins to anionic model membranes
2009
Abstract The role of electrostatics is studied in the adsorption of proteins to negatively charged (phosphatidylcholine/phosphatidylglycerol, PC/PG) and neutral (PC) small unilamellar vesicles (SUVs). For model proteins the interaction is monitored vs . pH at low ionic strength. The adsorption behaviour of lysozyme, myoglobin and albumin (isoelectronic point, p I 5–11) is investigated in SUVs, along with changes of the fluorescence emission spectra of the charged proteins, via their adsorption on SUVs. Significant adsorption of the proteins to negatively charged SUVs is found only at pH values, where the number of positive charge moieties exceeds the number of negative charge moieties on th…