Hb Helsinki: a variant with a high oxygen affinity and a substitution at a 2,3-DPG binding site (beta82[EF6] Lys replaced by Met). 1976

E Ikkala, and J Koskela, and P Pikkarainen, and E L Rahiala, and M A El-Hazmi, and K Nagai, and A Lang, and H Lehmann

A new haemoglobin, Hb Helsinki, in which beta 82-Lys (EF6) is replaced by Met, was found in a Finnish family. It was associated with familial erythrocytosis, and the oxygen affinity of the blood was higher than normal. The oxygen equilibrium curves of purified Hb Helsinki and HbA from the same haemolysate have been determined under vaious conditions. "Stripped' Hb Helsinki was found to show normal cooperativity, slightly low oxygen affinity and a reduced Bohr effect at physiological pH. However, the organic phosphates, 2,3-diphosphoglycerate (2,3-DPG) and inositol hexaphosphate (IHP) had a very small effect on Hb Helsinki, and the 2,3-DPG binding constant of deoxygenated Hb Helsinki is close to that of oxyhaemoglobin A. Thus, the replacement of Lys by Met at position 82 dramatically changes the nature of the central cavity of the tetramer and the effect of 2,3-DPG on the respiratory function of the molecule.

UI MeSH Term Description Entries
D008297 Male Males
D008715 Methionine A sulfur-containing essential L-amino acid that is important in many body functions. L-Methionine,Liquimeth,Methionine, L-Isomer,Pedameth,L-Isomer Methionine,Methionine, L Isomer
D008875 Middle Aged An adult aged 45 - 64 years. Middle Age
D010100 Oxygen An element with atomic symbol O, atomic number 8, and atomic weight [15.99903; 15.99977]. It is the most abundant element on earth and essential for respiration. Dioxygen,Oxygen-16,Oxygen 16
D010108 Oxyhemoglobins A compound formed by the combination of hemoglobin and oxygen. It is a complex in which the oxygen is bound directly to the iron without causing a change from the ferrous to the ferric state. Oxycobalt Hemoglobin,Oxycobalthemoglobin,Oxyhemoglobin,Hemoglobin, Oxycobalt
D010313 Partial Pressure The pressure that would be exerted by one component of a mixture of gases if it were present alone in a container. (From McGraw-Hill Dictionary of Scientific and Technical Terms, 6th ed) Partial Pressures,Pressure, Partial,Pressures, Partial
D010833 Phytic Acid Complexing agent for removal of traces of heavy metal ions. It acts also as a hypocalcemic agent. Inositol Hexaphosphate,Phytin,Calcium Phytate,Inositol Hexakisphosphate,Phytate,Sodium Phytate,Acid, Phytic,Hexakisphosphate, Inositol,Hexaphosphate, Inositol,Phytate, Calcium,Phytate, Sodium
D011086 Polycythemia An increase in the total red cell mass of the blood. (Dorland, 27th ed) Erythrocytosis,Erythrocytoses,Polycythemias
D011485 Protein Binding The process in which substances, either endogenous or exogenous, bind to proteins, peptides, enzymes, protein precursors, or allied compounds. Specific protein-binding measures are often used as assays in diagnostic assessments. Plasma Protein Binding Capacity,Binding, Protein
D004163 Diphosphoglyceric Acids Glyceric acids where two of the hydroxyl groups have been replaced by phosphates. Bisphosphoglycerates,Acids, Diphosphoglyceric

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