Search results for "Globin"
showing 10 items of 734 documents
Nonsense-mediated decay mechanism is a possible modifying factor of clinical outcome in nonsense cd39 beta thalassemia genotype
2012
Nonsense-mediated mRNA decay (NMD) is a surveillance system to prevent the synthesis of non-functional proteins. In β-thalassemia, NMD may have a role in clinical outcome. An example of premature translation stop codons appearing for the first time is the β-globin cd39 mutation; when homozygous, this results in a severe phenotype. The aim of this study was to determine whether the homozygous nonsense cd39 may have a milder phenotype in comparison with IVS1,nt110/cd39 genotype. Genotypes have been identified from a cohort of 568 patients affected by β-thalassemia. These genotypes were compared with those found in 577 affected fetuses detected among 2292 prenatal diagnoses. The…
Oxygen exchange in the erythrocyte
1969
Es wird eine Ubersicht uber die mathematischen und experimentellen Aspekte des O2-Austausches im Erythrozyten gegeben. Der Gesamtprozess kann als eine Sauerstoffdiffusion mit gekoppelter Hamoglobin-Sauerstoff-Reaktion beschrieben werden. Fur die mathematische Analyse muss also die partielle Differentialgleichung der Diffusion durch ein Gleichungssystem erweitert werden, das dem chemischen Reaktionsablauf Rechnung tragt. Voraussetzung hierfur ist die Aufstellung eines Modells zur molekularen Interpretation der O2-Bindungskurve. Die vereinfachten Differentialgleichungen fur Diffusion und Reaktion lassen sich unter Beachtung der Rand- und Anfangsbedingungen nur naherungsweise integrieren.
Oxidized respiratory molecules induced in vivo by nitrite in Pontodrilus littoralis (oligochaeta)
1992
Abstract 1. The in vivo effect of nitrite has been investigated in Pontodrilus littoralis. 2. Exposure of the animals at nitrite concentrations (0.05–0.1%) produced methaemoglobin and hemichromes. 3. Disappearance of oxidized forms was obtained when normal rearing conditions were restored. 4. Longer exposure to higher nitrite concentrations produced irreversible oxidized forms.
Calidad de la farmacoterapia y seguridad de los pacientes en hemodiálisis tratados con estimulantes eritropoyéticos
2008
Objective: To assess an interdisciplinary programme for the improvement of pharmacotherapy quality and patient safety in patients with chronic renal disease who are treated with erythropoiesis-stimulating agents. Method: Observational, longitudinal study. Retrospective analysis (period A) and prospective analysis (period B) following implementation of the programme for haemoglobin values and monthly erythropoiesis- stimulating agent dosage. The proportion of patients with haemoglobin values within the target range (10-5-12.5 g/dl) and those with values above the safety limit (≥ 12.5g/dl) were compared every four months and the average percentage of time with haemoglobin values within the ta…
Failure of 1H-[1,2,4]oxadiazolo[4,3-a]quinoxalin-1-one (ODQ) to inhibit soluble guanylyl cyclase in rat ventricular cardiomyocytes
1999
The effects of 1H-[1,2,4]oxadiazolo[4,3-a]quinoxalin-1-one (ODQ), an inhibitor of soluble guanylyl cyclase (sGC), were investigated in aortic rings and ventricular cardiomyocytes from rats. The production of cyclic GMP was stimulated by NO•-donors or carbachol. Additionally, the effects of ODQ were studied in cytosolic extracts from both tissues in which the cyclic GMP production was stimulated by S-nitroso-N-acetylpenicillamine (SNAP). In endothelium-intact aortic rings, SNAP (100 μM), 2,2′-(hydroxynitrosohydrazino)bis-ethanamine (DETA NONOate; 100 μM), or carbachol (10 μM) increased cyclic GMP levels about 4 fold. These effects were abolished by ODQ (50 μM). In cardiomyocytes, SNAP (100 μ…
Effects of nitric oxide donors on cardiac contractility in wild-type and myoglobin-deficient mice
2002
1. The effects of the nitric oxide (NO) donors S-nitroso-N-acetylpenicillamine (SNAP), sodium(Z)-1-(N,N-diethylamino)diazen-1-ium-1,2-diolate (DEA-NONOate), and (Z)-1-[N-(2-Aminoethyl)-N-(2-ammonioethyl)amino]diazen-1-ium-1,2-diolate (DETA-NONOate) on force of contraction (F(c)) were studied in atrial and ventricular muscle strips obtained from wild-type (WT) and myoglobin-deficient (myo(-/-)) mice. 2. SNAP slightly reduced F(c) in preparations from WT mice at concentrations above 100 microM; this effect was more pronounced in myo(-/-) mice. 3. DEA-NONOate reduced F(c) in preparations from myo(-/-) mice to a larger extent than those from WT mice. 4. DETA-NONOate reduced F(c) in preparations…
Time scale of protein aggregation dictated by liquid-liquid demixing
2003
The growing impact of protein aggregation pathologies, together with the current high need for extensive information on protein structures are focusing much interest on the physics underlying the nucleation and growth of protein aggregates and crystals. Sickle Cell Hemoglobin (HbS), a point-mutant form of normal human Hemoglobin (HbA), is the first recognized and best-studied case of pathologically aggregating protein. Here we reanalyze kinetic data on nucleation of deoxy-HbS aggregates by referring them to the (concentration-dependent) temperature Ts characterizing the occurrence of the phase transition of liquid-liquid demixing (LLD) of the solution. In this way, and by appropriate scalin…
Unveiling the timescale of the R-T transition in human hemoglobin.
2010
Time-resolved wide-angle X-ray scattering, a recently developed technique allowing to probe global structural changes of proteins in solution, was used to investigate the kinetics of R-T quaternary transition in human hemoglobin and to systematically compare it to that obtained with time-resolved optical spectroscopy under nearly identical experimental conditions. Our data reveal that the main structural rearrangement associated with the R-T transition takes place approximately 2 mus after the photolysis of hemoglobin at room temperature and neutral pH. This finding suggests that the 20-mus step observed with time-resolved optical spectroscopy corresponds to a small and localized structural…
Observing heme doming in myoglobin with femtosecond X-ray absorption spectroscopy.
2015
International audience; We report time-resolved X-ray absorption measurements after photolysis of carbonmonoxy myoglobin performed at the LCLS X-ray free electron laser with nearly 100 fs (FWHM) time resolution. Data at the Fe K-edge reveal that the photoinduced structural changes at the heme occur in two steps, with a faster (∼70 fs) relaxation preceding a slower (∼400 fs) one. We tentatively attribute the first relaxation to a structural rearrangement induced by photolysis involving essentially only the heme chromophore and the second relaxation to a residual Fe motion out of the heme plane that is coupled to the displacement of myoglobin F-helix
X-ray structure determination of a metastable state of carbonmonoxy myoglobin after photodissociation.
1996
The x-ray structure of carbon monoxide (CO)-ligated myoglobin illuminated during data collection by a laser diode at the wavelength lambda = 690 nm has been determined to a resolution of 1.7 A at T = 36 K. For comparison, we also measured data sets of deoxymyoglobin and CO-ligated myoglobin. In the photon-induced structure the electron density associated with the CO ligand can be described by a tube extending from the iron into the heme pocket over more than 4 A. This density can be interpreted by two discrete positions of the CO molecule. One is close to the heme iron and can be identified to be bound CO. In the second, the CO is dissociated from the heme iron and lies on top of pyrrole ri…