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1r1y

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|RESOURCES=<span class='plainlinks'>[http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=1r1y FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=1r1y OCA], [http://www.ebi.ac.uk/pdbsum/1r1y PDBsum], [http://www.rcsb.org/pdb/explore.do?structureId=1r1y RCSB]</span>
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'''Crystal structure of deoxy-human hemoglobin Bassett at 1.8 angstrom'''
'''Crystal structure of deoxy-human hemoglobin Bassett at 1.8 angstrom'''
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[[Category: Ochotorena, J.]]
[[Category: Ochotorena, J.]]
[[Category: Safo, M K.]]
[[Category: Safo, M K.]]
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[[Category: crystal structure]]
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[[Category: Crystal structure]]
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[[Category: deoxyhemoglobin]]
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[[Category: Deoxyhemoglobin]]
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[[Category: mutant]]
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[[Category: Mutant]]
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[[Category: oxygen affinity]]
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[[Category: Oxygen affinity]]
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[[Category: rochester]]
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Revision as of 03:58, 3 May 2008

Template:STRUCTURE 1r1y

Crystal structure of deoxy-human hemoglobin Bassett at 1.8 angstrom


Overview

Hemoglobin (Hb) Bassett, an abnormal Hb variant with a markedly reduced oxygen affinity, was discovered in a Caucasian (Anglo-Saxon) male child who experienced episodes of cyanosis. Cation-exchange and reversed-phase (RP) high-performance liquid chromatography (HPLC) showed that the patient has an abnormal Hb, with a mutation in the alpha-globin. Tryptic peptide digest of the abnormal alpha-globin with subsequent HPLC analysis revealed abnormal elution of the alpha-T11 peptide. Further studies with Edman sequencing and electrospray mass spectrometry of tryptic peptide alpha-T11, as well as structural analysis by X-ray crystallography revealed an Asp-->Ala substitution at the alpha94 (G1) position, a match for Hb Bassett. Detailed functional studies showed that this Hb variant had a markedly reduced oxygen affinity (P(50) at pH 7.0 = 22 mmHg; Hb A P(50) = 10.5 mmHg), reduced Bohr effect (-0.26 compared to - 0.54 in Hb A), and low subunit cooperativity (n = 1.4, compared to 2.6 in Hb A). X-ray crystallography results explain the probable effects of the structural modification on the oxygen-binding properties of this Hb variant.

About this Structure

1R1Y is a Protein complex structure of sequences from Homo sapiens. Full crystallographic information is available from OCA.

Reference

Characterization of hemoglobin bassett (alpha94Asp-->Ala), a variant with very low oxygen affinity., Abdulmalik O, Safo MK, Lerner NB, Ochotorena J, Daikhin E, Lakka V, Santacroce R, Abraham DJ, Asakura T, Am J Hematol. 2004 Nov;77(3):268-76. PMID:15495251 Page seeded by OCA on Sat May 3 06:58:36 2008

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