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  • molecule
  • Stereo-specific binding of isopropyl group of βVal6, the mutated amino-acid residue of a tetrameric sickle hemoglobin molecule (HbS), with hydrophobic groove of another HbS tetramer initiates the polymerization. (biochemj.org)
  • trait
  • These maps show the worldwide distribution of the mutation responsible for the sickle cell trait (a and b) and of malaria incidence (c). (labspaces.net)
  • However, it was found that these same individuals, said to carry the sickle cell trait, were in fact highly protected against malaria, thus explaining the high prevalence of this mutation in geographical areas where malaria is endemic. (labspaces.net)
  • In painstakingly detailed work, Ana Ferreira, a post-doctoral researcher in Miguel Soares' laboratory, demonstrated that mice obtained from Prof. Yves Beuzard's laboratory, that had been genetically engineered to produce one copy of sickle hemoglobin similar to sickle cell trait, do not succumb to cerebral malaria, thus reproducing what happens in humans. (labspaces.net)
  • About 4% of study subjects had co-existence of sickle cell Hb trait and G6PD deficiency. (biomedcentral.com)
  • There was no significant influence of the sickle cell trait on malaria incidence among older children of 1-9 years. (biomedcentral.com)
  • genetic
  • Through a series of genetic experiments, Ana Ferreira was able to show that the main player in this protective effect is heme oxygenase-1 (HO-1), an enzyme whose expression is strongly induced by sickle hemoglobin. (labspaces.net)
  • Since malaria infects red blood cells, these genetic changes are most commonly alterations to molecules essential for red blood cell function (and therefore parasite survival), such as hemoglobin or other cellular proteins or enzymes of red blood cells. (wikipedia.org)
  • hydrophobic
  • Urea is proposed to act reversibly by interfering with the hydrophobic bonding of hemoglobin molecules, (Murayama, 1971), while cyanate is reported to act irreversibly through the carbamyl ation of the amino terminal valines, (Cerami and Manning, 1971). (springer.com)
  • oxygen
  • People who have SCD have a different type of hemoglobin (protein that carries oxygen). (bioportfolio.com)
  • When a sufferer's red blood cells are exposed to low-oxygen conditions, the cells lose their healthy round shape and become sickle-shaped. (wikipedia.org)
  • patients
  • 47 patients hemoglobin SS aged 5-42 years, on regular follow up in Military hospital were recruited from April 2014 to January 2015 Patients received dose of G A 30g/day for 12 weeks. (clinicaltrials.gov)
  • Patients may receive hydroxyurea to induce the protective effects of increased fetal hemoglobin production. (wikipedia.org)
  • chains
  • A solvent-free coarse-grained molecular dynamics (CGMD) model has been developed and it represents a single hemoglobin fiber with four tightly bonded chains, each of which comprises soft particles. (asme.org)
  • blood
  • Ana Ferreira went on to show that the protection afforded by sickle hemoglobin in these mice, acts without interfering directly with the parasite's ability to infect the host red blood cells. (labspaces.net)
  • red blood cells to change their shape from a round and doughnut-like shape to a half-moon/crescent, or sickled shape. (bioportfolio.com)
  • This different type of hemoglobin makes the red blood cells change into a crescent shape under certain conditions. (bioportfolio.com)
  • Sickle-shaped cells are a problem because they often get stuck in the blood vessels blocking the flow of blood and can cause inflammation and injury to important areas of the body. (bioportfolio.com)
  • Hemoglobin function in stored blood. (springer.com)
  • Increased deoxygenation causes sickling of red blood cells, which adhere to the spleen wall and splenic macrophages causing ischemia. (wikipedia.org)
  • Caltech THESIS : A Caltech Library Service Early Hemoglobin Investigations, It's in the Blood! (wikipedia.org)
  • populations
  • Observations made during the mid-20th century and building on Pauling's findings, revealed that the sickle mutation is, in fact, highly, selected in populations from areas of the world were malaria is very frequent, with sometimes 10-40% of the population carrying this mutation. (labspaces.net)
  • cell anaemia
  • in 2014 16,247 people were tested, 342 were Beta-thalassaemia carriers, 8 had sickle-cell anaemia, 205 were sickle-cell anaemia carriers, 36 had syphilis and 140 had hepatitis B. In 1985 the UAE established a national program to prevent transmission of acquired immune deficiency syndrome (AIDS) and to control its entry into the country. (wikipedia.org)
  • RBCs
  • Beyond the mutation of Hb as the cause of SCD, Hebbel et al initiated a new era of research in 1980 by introducing the concept that sickle red blood cells (RBCs) are abnormally adhesive. (blogspot.fi)
  • Many subsequent studies from Hebbel et al and others led to the realization that not only sickle RBCs but other blood cells, especially leukocytes and platelets, are activated and have the potential to contribute to vaso-occlusive crises. (blogspot.fi)
  • cystic
  • Cystic fibrosis carrier screening, chromosome analyses, and hemoglobin evaluations are performed on the majority of sperm donor applicants in the United States. (wikipedia.org)
  • hydroxyurea
  • Despite these early discoveries, the life expectancy of sickle cell patients only began to improve significantly within the last 30 years, first with the introduction of prophylactic penicillin V in the 1980s, followed by more aggressive blood transfusions, and in 1998, with the introduction of hydroxyurea as a mainstay of treatment. (blogspot.fi)
  • amino
  • The globin portion of hemoglobin is degraded to its constitutive amino acids, and the heme portion is metabolized to bilirubin, which is removed in the liver. (wikipedia.org)
  • valine
  • Details of the mutation (replacement of glutamic acid with valine in the 6th position of the hemoglobin [Hb] β-chain) were first described by Ingram in 1956. (blogspot.fi)
  • Because valine is hydrophobic, whereas glutamic acid is hydrophilic, this change makes the hemoglobin prone to abnormal aggregation. (wikipedia.org)
  • crisis
  • Without a history of exposure, lead poisoning has been mistaken for acute viral illness, sickle cell vasoocclusive crisis, gastro-enteritis, acute appendicitis, nephrolithiasis, and Guillain-Barré syndrome. (aafp.org)
  • proteins
  • Perutz discovered that the detailed three-dimensional structures of proteins, such as myoglobin and hemoglobin could, in principle, be solved by X-ray analysis using a heavy metal atom labeling technique. (wikipedia.org)
  • acid
  • Another point mutation in HBB, in which glutamic acid is replaced with lysine at position 26 (β26Glu→Lys), leads to the formation of haemoglobin E (HbE). (wikipedia.org)
  • species
  • Alpha-hemolytic species, including S. pneumoniae, Streptococcus mitis, S. mutans, and S. salivarius, oxidize the iron in the hemoglobin (turning it dark green in culture). (wikipedia.org)
  • increase
  • Haemolysis: Will falsely increase the MCHC (measured haemoglobin is proportionally higher than HCT or PCV) and falsely decrease the calculated haemoglobin (fewer intact RBC) Other: Heinz bodies (many, particularly if large) may falsely increase the MCHC but not the MCH. (wikipedia.org)
  • cells
  • The Mean corpuscular hemoglobin concentration, a measure of the concentration of haemoglobin in a given volume of packed red blood cells. (wikipedia.org)
  • MCHC can be falsely elevated when there is agglutination of red cells (falsely lowering the measured RBC) or when there is opacifaction of the plasma (falsely increasing the measured hemoglobin). (wikipedia.org)
  • As a part of the mononuclear phagocyte system, it metabolizes hemoglobin removed from senescent red blood cells (erythrocytes). (wikipedia.org)
  • long
  • His first technical paper, a 30-page long article on haemoglobin function, was published that same year, as a co-author alongside his father. (wikipedia.org)