1nih

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spinqualitylabelsall models 1nih, resolution 2.6Å Show:Asymmetric Unit Biological Assembly Drag the structure with the mouse to rotate   Export Animated Image

Structure of deoxy-quaternary haemoglobin with liganded beta subunits

Contents 1 Overview 2 Disease 3 About this Structure 4 Reference

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 , , and 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

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