1nqp: Difference between revisions
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New page: left|200px<br /> <applet load="1nqp" size="450" color="white" frame="true" align="right" spinBox="true" caption="1nqp, resolution 1.73Å" /> '''Crystal structure o... |
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==Overview== | ==Overview== | ||
Hemoglobin A(2) (alpha(2)delta(2)), a minor (2-3%) component of, circulating red blood cells, acts as an anti-sickling agent and its, elevated concentration in beta-thalassemia is a useful clinical, diagnostic. In beta-thalassemia major, where there is a failure of, beta-chain production, HbA(2) acts as the predominant oxygen delivery, mechanism. Hemoglobin E, is another common abnormal hemoglobin, caused by, splice site mutation in exon 1 of beta globin gene, when combines with, beta-thalassemia, causes severe microcytic anemia. The purification, crystallization, and preliminary structural studies of HbA(2) and HbE are, reported here. HbA(2) and HbE are purified by cation exchange column, chromatography in presence of KCN from the blood samples of individuals, suffering from beta-thalassemia minor and E beta-thalassemia. X-ray, diffraction data of HbA(2) and HbE were collected upto 2.1 and 1.73 A, respectively. HbA(2) crystallized in space group P2(1) with unit cell, parameters a=54.33 A, b=83.73 A, c=62.87 A, and beta=99.80 degrees whereas, HbE crystallized in space group P2(1)2(1)2(1) with unit cell parameters, a=60.89 A, b=95.81 A, and c=99.08 A. Asymmetric unit in each case contains, one Hb tetramer in R(2) state. | Hemoglobin A(2) (alpha(2)delta(2)), a minor (2-3%) component of, circulating red blood cells, acts as an anti-sickling agent and its, elevated concentration in beta-thalassemia is a useful clinical, diagnostic. In beta-thalassemia major, where there is a failure of, beta-chain production, HbA(2) acts as the predominant oxygen delivery, mechanism. Hemoglobin E, is another common abnormal hemoglobin, caused by, splice site mutation in exon 1 of beta globin gene, when combines with, beta-thalassemia, causes severe microcytic anemia. The purification, crystallization, and preliminary structural studies of HbA(2) and HbE are, reported here. HbA(2) and HbE are purified by cation exchange column, chromatography in presence of KCN from the blood samples of individuals, suffering from beta-thalassemia minor and E beta-thalassemia. X-ray, diffraction data of HbA(2) and HbE were collected upto 2.1 and 1.73 A, respectively. HbA(2) crystallized in space group P2(1) with unit cell, parameters a=54.33 A, b=83.73 A, c=62.87 A, and beta=99.80 degrees whereas, HbE crystallized in space group P2(1)2(1)2(1) with unit cell parameters, a=60.89 A, b=95.81 A, and c=99.08 A. Asymmetric unit in each case contains, one Hb tetramer in R(2) state. | ||
==Disease== | |||
Known diseases associated with this structure: Erythremias, alpha- OMIM:[[http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=141800 141800]], Erythremias, beta- OMIM:[[http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=141900 141900]], Erythrocytosis OMIM:[[http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=141850 141850]], HPFH, deletion type OMIM:[[http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=141900 141900]], Heinz body anemia OMIM:[[http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=141850 141850]], Heinz body anemias, alpha- OMIM:[[http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=141800 141800]], Heinz body anemias, beta- OMIM:[[http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=141900 141900]], Hemoglobin H disease OMIM:[[http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=141850 141850]], Hypochromic microcytic anemia OMIM:[[http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=141850 141850]], Methemoglobinemias, alpha- OMIM:[[http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=141800 141800]], Methemoglobinemias, beta- OMIM:[[http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=141900 141900]], Sickle cell anemia OMIM:[[http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=141900 141900]], Thalassemia, alpha- OMIM:[[http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=141850 141850]], Thalassemia-beta, dominant inclusion-body OMIM:[[http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=141900 141900]], Thalassemias, alpha- OMIM:[[http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=141800 141800]], Thalassemias, beta- OMIM:[[http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=141900 141900]] | |||
==About this Structure== | ==About this Structure== | ||
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[[Category: hemoglobin e oxygen transport beta thalassemia]] | [[Category: hemoglobin e oxygen transport beta thalassemia]] | ||
''Page seeded by [http://ispc.weizmann.ac.il/oca OCA ] on | ''Page seeded by [http://ispc.weizmann.ac.il/oca OCA ] on Mon Nov 12 18:24:10 2007'' | ||
