1r1x

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[[Image:1r1x.gif|left|200px]]<br /><applet load="1r1x" size="350" color="white" frame="true" align="right" spinBox="true"
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[[Image:1r1x.gif|left|200px]]
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caption="1r1x, resolution 2.15&Aring;" />
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'''Crystal structure of oxy-human hemoglobin Bassett at 2.15 angstrom'''<br />
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{{Structure
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|PDB= 1r1x |SIZE=350|CAPTION= <scene name='initialview01'>1r1x</scene>, resolution 2.15&Aring;
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|SITE=
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|LIGAND= <scene name='pdbligand=CMO:CARBON+MONOXIDE'>CMO</scene>, <scene name='pdbligand=HEM:PROTOPORPHYRIN+IX+CONTAINING+FE'>HEM</scene> and <scene name='pdbligand=MBN:TOLUENE'>MBN</scene>
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|ACTIVITY=
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|GENE=
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}}
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'''Crystal structure of oxy-human hemoglobin Bassett at 2.15 angstrom'''
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==Overview==
==Overview==
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==About this Structure==
==About this Structure==
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1R1X is a [http://en.wikipedia.org/wiki/Protein_complex Protein complex] structure of sequences from [http://en.wikipedia.org/wiki/Homo_sapiens Homo sapiens] with <scene name='pdbligand=CMO:'>CMO</scene>, <scene name='pdbligand=HEM:'>HEM</scene> and <scene name='pdbligand=MBN:'>MBN</scene> as [http://en.wikipedia.org/wiki/ligands ligands]. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=1R1X OCA].
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1R1X is a [[Protein complex]] structure of sequences from [http://en.wikipedia.org/wiki/Homo_sapiens Homo sapiens]. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=1R1X OCA].
==Reference==
==Reference==
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Characterization of hemoglobin bassett (alpha94Asp--&gt;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:[http://ispc.weizmann.ac.il//pmbin/getpm?pmid=15495251 15495251]
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Characterization of hemoglobin bassett (alpha94Asp--&gt;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:[http://www.ncbi.nlm.nih.gov/pubmed/15495251 15495251]
[[Category: Homo sapiens]]
[[Category: Homo sapiens]]
[[Category: Protein complex]]
[[Category: Protein complex]]
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[[Category: rochester]]
[[Category: rochester]]
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''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on Thu Feb 21 14:46:08 2008''
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''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on Thu Mar 20 13:45:45 2008''

Revision as of 11:45, 20 March 2008

Image:1r1x.gif


PDB ID 1r1x

Drag the structure with the mouse to rotate
, resolution 2.15Å
Ligands: , and
Coordinates: save as pdb, mmCIF, xml



Crystal structure of oxy-human hemoglobin Bassett at 2.15 angstrom


Contents

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.

Disease

Known diseases associated with this structure: Erythremias, alpha- OMIM:[141800], Erythremias, beta- OMIM:[141900], Erythrocytosis OMIM:[141850], HPFH, deletion type OMIM:[141900], Heinz body anemia OMIM:[141850], Heinz body anemias, alpha- OMIM:[141800], Heinz body anemias, beta- OMIM:[141900], Hemoglobin H disease OMIM:[141850], Hypochromic microcytic anemia OMIM:[141850], Methemoglobinemias, alpha- OMIM:[141800], Methemoglobinemias, beta- OMIM:[141900], Sickle cell anemia OMIM:[141900], Thalassemia, alpha- OMIM:[141850], Thalassemia-beta, dominant inclusion-body OMIM:[141900], Thalassemias, alpha- OMIM:[141800], Thalassemias, beta- OMIM:[141900]

About this Structure

1R1X 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

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