1nih
From Proteopedia
(New page: 200px<br /> <applet load="1nih" size="450" color="white" frame="true" align="right" spinBox="true" caption="1nih, resolution 2.6Å" /> '''Structure of deoxy-q...) |
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==Overview== | ==Overview== | ||
We have determined the structure of a T-state haemoglobin in which the, haem groups of the beta subunits have carbon monoxide bound, and the alpha, subunits have nickel replacing the haem iron and are ligand-free. The, structural adjustments on binding ligand in the T state are in the same, direction as those associated with the quaternary transition, and a, translational shift of the haem is severely restricted. We explain how, these observations may account for the low ligand affinity of the beta, haem of T-state haemoglobin. | We have determined the structure of a T-state haemoglobin in which the, haem groups of the beta subunits have carbon monoxide bound, and the alpha, subunits have nickel replacing the haem iron and are ligand-free. The, structural adjustments on binding ligand in the T state are in the same, direction as those associated with the quaternary transition, and a, translational shift of the haem is severely restricted. We explain how, these observations may account for the low ligand affinity of the beta, haem of T-state haemoglobin. | ||
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| + | ==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: oxygen transport]] | [[Category: oxygen transport]] | ||
| - | ''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 18:21:54 2007'' |
Revision as of 16:15, 12 November 2007
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Structure of deoxy-quaternary haemoglobin with liganded beta subunits
Contents |
Overview
We have determined the structure of a T-state haemoglobin in which the, haem groups of the beta subunits have carbon monoxide bound, and the alpha, subunits have nickel replacing the haem iron and are ligand-free. The, structural adjustments on binding ligand in the T state are in the same, direction as those associated with the quaternary transition, and a, translational shift of the haem is severely restricted. We explain how, these observations may account for the low ligand affinity of the beta, haem of T-state haemoglobin.
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
1NIH is a Protein complex structure of sequences from Homo sapiens with HNI, HEM, CMO and IHP as ligands. Full crystallographic information is available from OCA.
Reference
Structure of deoxy-quaternary haemoglobin with liganded beta subunits., Luisi B, Liddington B, Fermi G, Shibayama N, J Mol Biol. 1990 Jul 5;214(1):7-14. PMID:2370669
Page seeded by OCA on Mon Nov 12 18:21:54 2007
Categories: Homo sapiens | Protein complex | Liddington, B. | Luisi, B. | CMO | HEM | HNI | IHP | Oxygen transport
