1hrk
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(New page: 200px<br /> <applet load="1hrk" size="450" color="white" frame="true" align="right" spinBox="true" caption="1hrk, resolution 2.00Å" /> '''CRYSTAL STRUCTURE O...)
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Revision as of 15:15, 12 November 2007
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CRYSTAL STRUCTURE OF HUMAN FERROCHELATASE
Contents |
Overview
Human ferrochelatase (E.C. 4.99.1.1) is a homodimeric (86 kDa), mitochondrial membrane-associated enzyme that catalyzes the insertion of, ferrous iron into protoporphyrin to form heme. We have determined the 2.0, A structure from the single wavelength iron anomalous scattering signal., The enzyme contains two NO-sensitive and uniquely coordinated [2Fe-2S], clusters. Its membrane association is mediated in part by a 12-residue, hydrophobic lip that also forms the entrance to the active site pocket., The positioning of highly conserved residues in the active site in, conjunction with previous biochemical studies support a catalytic model, that may have significance in explaining the enzymatic defects that lead, to the human inherited disease erythropoietic protoporphyria.
Disease
Known diseases associated with this structure: Protoporphyria, erythropoietic OMIM:[177000], Protoporphyria, erythropoietic, recessive, with liver failure OMIM:[177000]
About this Structure
1HRK is a Single protein structure of sequence from Homo sapiens with CHD and FES as ligands. Active as Ferrochelatase, with EC number 4.99.1.1 Full crystallographic information is available from OCA.
Reference
The 2.0 A structure of human ferrochelatase, the terminal enzyme of heme biosynthesis., Wu CK, Dailey HA, Rose JP, Burden A, Sellers VM, Wang BC, Nat Struct Biol. 2001 Feb;8(2):156-60. PMID:11175906
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