1nej

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[[Image:1nej.gif|left|200px]]<br /><applet load="1nej" size="350" color="white" frame="true" align="right" spinBox="true"
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[[Image:1nej.gif|left|200px]]
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caption="1nej, resolution 2.10&Aring;" />
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'''Crystalline Human Carbonmonoxy Hemoglobin S (Liganded Sickle Cell Hemoglobin) Exhibits The R2 Quaternary State At Neutral pH In The Presence Of Polyethylene Glycol: The 2.1 Angstrom Resolution Crystal Structure'''<br />
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{{Structure
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|PDB= 1nej |SIZE=350|CAPTION= <scene name='initialview01'>1nej</scene>, resolution 2.10&Aring;
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|SITE=
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|LIGAND= <scene name='pdbligand=HEM:PROTOPORPHYRIN+IX+CONTAINING+FE'>HEM</scene> and <scene name='pdbligand=CMO:CARBON MONOXIDE'>CMO</scene>
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|ACTIVITY=
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|GENE=
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}}
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'''Crystalline Human Carbonmonoxy Hemoglobin S (Liganded Sickle Cell Hemoglobin) Exhibits The R2 Quaternary State At Neutral pH In The Presence Of Polyethylene Glycol: The 2.1 Angstrom Resolution Crystal Structure'''
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==Overview==
==Overview==
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==About this Structure==
==About this Structure==
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1NEJ 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=HEM:'>HEM</scene> and <scene name='pdbligand=CMO:'>CMO</scene> as [http://en.wikipedia.org/wiki/ligands ligands]. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=1NEJ OCA].
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1NEJ 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=1NEJ OCA].
==Reference==
==Reference==
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COHbC and COHbS crystallize in the R2 quaternary state at neutral pH in the presence of PEG 4000., Patskovska LN, Patskovsky YV, Almo SC, Hirsch RE, Acta Crystallogr D Biol Crystallogr. 2005 May;61(Pt 5):566-73. Epub 2005, Apr 20. PMID:[http://ispc.weizmann.ac.il//pmbin/getpm?pmid=15858266 15858266]
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COHbC and COHbS crystallize in the R2 quaternary state at neutral pH in the presence of PEG 4000., Patskovska LN, Patskovsky YV, Almo SC, Hirsch RE, Acta Crystallogr D Biol Crystallogr. 2005 May;61(Pt 5):566-73. Epub 2005, Apr 20. PMID:[http://www.ncbi.nlm.nih.gov/pubmed/15858266 15858266]
[[Category: Homo sapiens]]
[[Category: Homo sapiens]]
[[Category: Protein complex]]
[[Category: Protein complex]]
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[[Category: mutant human hemoglobin s[betae6v]; r2 quaternary state; human hemoglobin]]
[[Category: mutant human hemoglobin s[betae6v]; r2 quaternary state; human hemoglobin]]
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''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on Thu Feb 21 14:05:14 2008''
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''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on Thu Mar 20 12:55:51 2008''

Revision as of 10:55, 20 March 2008


PDB ID 1nej

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, resolution 2.10Å
Ligands: and
Coordinates: save as pdb, mmCIF, xml



Crystalline Human Carbonmonoxy Hemoglobin S (Liganded Sickle Cell Hemoglobin) Exhibits The R2 Quaternary State At Neutral pH In The Presence Of Polyethylene Glycol: The 2.1 Angstrom Resolution Crystal Structure


Contents

Overview

Human hemoglobin binds oxygen cooperatively and functions as a tetramer composed of two identical alphabeta heterodimers. While human hemoglobin is the best characterized allosteric protein, the quaternary R (oxygenated or liganded) to T (deoxygenated) structural transition remains controversial. The R2 state has been postulated to represent either an intermediate or final quaternary state elicited by ligand binding. However, the biological relevance of the R2 state has been questioned as it has not been observed crystallographically under physiological conditions. The high-resolution R2 quaternary structures of human COHbC (betaE6K) and COHbS (betaE6V) are reported at neutral pH and low ionic strength using PEG 4000 as a precipitant. Crystals of COHbC, COHbS and their mixtures are isomorphous, indicating that they share the same tertiary and quaternary structures. In contrast, oxyHbA or COHbA did not yield crystals at neutral pH under similar conditions. Solubility studies and modeling suggest that at neutral pH and low ionic strength the beta6 mutant hemoglobins crystallize (betaK6 > betaV6) as a result of more favorable lattice contacts.

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

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

Reference

COHbC and COHbS crystallize in the R2 quaternary state at neutral pH in the presence of PEG 4000., Patskovska LN, Patskovsky YV, Almo SC, Hirsch RE, Acta Crystallogr D Biol Crystallogr. 2005 May;61(Pt 5):566-73. Epub 2005, Apr 20. PMID:15858266[[Category: mutant human hemoglobin s[betae6v]; r2 quaternary state; human hemoglobin]]

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