Search results for "globin"

Anemie emolitiche da anomalie ereditarie dell'Hb.

1956

SUMMARYNew researches have made it evident that besides the two normal Hb, the adult one (a) and the phoetal (f), there are other pathologic Hb:c, e, s, d, g, h, i.These Hb, excepting theh, are present in the hetherozygote on every healthy person and in the homozygote, excepting thegHb, determine chronic hemolitic anemia.ThefHb, or a very similar Hb, is present in the Thalassemia minor and major, in some homozygote cases of abnormal Hb, in the interreaction between two different genes of these Hb, or between one of these genes with the one of Thalassemia.We may retain that the genesa, c, s, d, e, g, i, form an allelomorphic series. The geneThis not a part of this series.Since in the homozyg…

Study of some serum group systems in the Mahishyas and the Muslims in 24-Parganas district, West Bengal

1974

A survey of serum Pi, Cp, Hp and Tf was carried out in 104 Bengali Hindu Mahishya and 123 Bengali Muslim of West Bengal, India.

Role of Solvent on Protein-Matrix Coupling in MbCO Embedded in Water-Saccharide Systems: A Fourier Transform Infrared Spectroscopy Study

2006

AbstractEmbedding protein in sugar systems of low water content enables one to investigate the protein dynamic-structure function in matrixes whose rigidity is modulated by varying the content of residual water. Accordingly, studying the dynamics and structure thermal evolution of a protein in sugar systems of different hydration constitutes a tool for disentangling solvent rigidity from temperature effects. Furthermore, studies performed using different sugars may give information on how the detailed composition of the surrounding solvent affects the internal protein dynamics and structural evolution. In this work, we compare Fourier transform infrared spectroscopy measurements (300–20K) o…

The Interdependence of Respiratory Gas Values and pH as a Function of Base Excess in Human Blood at 37°C

1973

The accurate estimation of the transport function of human blood for respiratory gases as well as the respiratory gas exchange in human body requires detailed knowledge of the respiratory gas partial pressures and the acid-base status in the blood. Because there is an interdependence between the different respiratory gas parameters described as Bohr and Haldane effect (3,4), it is possible to obtain a general picture of the whole transport system, if these interrelations are known.

Hydration dependent dynamics in sol-gel encapsulated myoglobin.

2008

In this work we study the effect of hydration on the dynamics of a protein in confined geometry, i.e. encapsulated in a porous silica matrix. Using elastic neutron scattering we investigate the temperature dependence of the mean square displacements of non-exchangeable hydrogen atoms of sol-gel encapsulated met-myoglobin. The study is extended to samples at 0.2, 0.3 and 0.5 g water/g protein fractions and comparison is made with met-myoglobin powders at the same average hydration and with a dry powder sample. Elastic data are analysed using a model of dynamical heterogeneity to take into account deviations of elastic intensity from gaussian behaviour in a large momentum transfer range and r…

Thermal evolution of the CO stretching band in carboxy-myoglobin in the light of neutron scattering and molecular dynamics simulations

2008

Abstract As it is well known, the thermal behaviour of the CO stretching band in MbCO reflects the interconversion among protein’s taxonomic and lower tier substates. We compare here FTIR data on the thermal behaviour of the CO stretching band in MbCO embedded in non-liquid, water–trehalose matrixes, and neutron scattering data on dry and hydrated proteins and nucleic acids. The comparison, also in the light of simulative data, gives relevant information on the relationship between the mean square displacements of hydrogen atoms and the heme pocket thermal rearrangements in MbCO, as experienced by the bound CO, in the temperature region 100–200 K, and at higher temperature when large scale …

An Unexplained Congenital Disorder of Glycosylation-II in a Child with Neurohepatic Involvement, Hypercholesterolemia and Hypoceruloplasminemia

2017

We report on a 12-year-old adopted boy with psychomotor disability, absence seizures, and normal brain MRI. He showed increased (but initially, at 5 months, normal) serum cholesterol, increased alkaline phosphatases, transiently increased transaminases and hypoceruloplasminemia with normal serum and urinary copper. Blood levels of immunoglobulins, haptoglobin, antithrombin, and factor XI were normal. A type 2 serum transferrin isoelectrofocusing and hypoglycosylation of apoCIII pointed to a combined N- and O-glycosylation defect. Neither CDG panel analysis with 79 CDG-related genes, nor whole exome sequencing revealed the cause of this CDG. Whole genome sequencing was not performed since th…

Marked impact of IL28B genotype in the natural clearance of hepatitis C virus in patients with haemoglobinopathies.

2011

Respiratory gas exchange in the rat spleen in situ and intrasplenic oxyhemoglobin saturation.

1979

Measurements of splenic respiratory gas exchange and of HbO2 saturations in the red pulp of the rat spleen have shown that there are no indications of a reduced intrasplenic O2 availability during normoxia. The present studies provide evidence that, in the normal spleen, the intrasplenic sequestration of red blood cells cannot be explained by an O2 deficiency in the red pulp since the commonly accepted notion of an intrasplenic hypoxia is not true.

HSP70 sequestration by free α-globin promotes ineffective erythropoiesis in β-thalassaemia

2014

International audience; β-Thalassaemia major (β-TM) is an inherited haemoglobinopathy caused by a quantitative defect in the synthesis of β-globin chains of haemoglobin, leading to the accumulation of free α-globin chains that form toxic aggregates. Despite extensive knowledge of the molecular defects causing β-TM, little is known of the mechanisms responsible for the ineffective erythropoiesis observed in the condition, which is characterized by accelerated erythroid differentiation, maturation arrest and apoptosis at the polychromatophilic stage. We have previously demonstrated that normal human erythroid maturation requires a transient activation of caspase-3 at the later stages of matur…