1hrk
From Proteopedia
(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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| - | [[Image:1hrk.gif|left|200px]]<br /> | + | [[Image:1hrk.gif|left|200px]]<br /><applet load="1hrk" size="350" color="white" frame="true" align="right" spinBox="true" |
| - | <applet load="1hrk" size=" | + | |
caption="1hrk, resolution 2.00Å" /> | caption="1hrk, resolution 2.00Å" /> | ||
'''CRYSTAL STRUCTURE OF HUMAN FERROCHELATASE'''<br /> | '''CRYSTAL STRUCTURE OF HUMAN FERROCHELATASE'''<br /> | ||
==Overview== | ==Overview== | ||
| - | Human ferrochelatase (E.C. 4.99.1.1) is a homodimeric (86 kDa) | + | 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== | ==Disease== | ||
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==About this Structure== | ==About this Structure== | ||
| - | 1HRK is a [http://en.wikipedia.org/wiki/Single_protein Single protein] structure of sequence from [http://en.wikipedia.org/wiki/Homo_sapiens Homo sapiens] with CHD and FES as [http://en.wikipedia.org/wiki/ligands ligands]. Active as [http://en.wikipedia.org/wiki/Ferrochelatase Ferrochelatase], with EC number [http://www.brenda-enzymes.info/php/result_flat.php4?ecno=4.99.1.1 4.99.1.1] Full crystallographic information is available from [http:// | + | 1HRK is a [http://en.wikipedia.org/wiki/Single_protein Single protein] structure of sequence from [http://en.wikipedia.org/wiki/Homo_sapiens Homo sapiens] with <scene name='pdbligand=CHD:'>CHD</scene> and <scene name='pdbligand=FES:'>FES</scene> as [http://en.wikipedia.org/wiki/ligands ligands]. Active as [http://en.wikipedia.org/wiki/Ferrochelatase Ferrochelatase], with EC number [http://www.brenda-enzymes.info/php/result_flat.php4?ecno=4.99.1.1 4.99.1.1] Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=1HRK OCA]. |
==Reference== | ==Reference== | ||
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[[Category: Single protein]] | [[Category: Single protein]] | ||
[[Category: Burden, A.]] | [[Category: Burden, A.]] | ||
| - | [[Category: Dailey, H | + | [[Category: Dailey, H A.]] |
| - | [[Category: Rose, J | + | [[Category: Rose, J P.]] |
| - | [[Category: Sellers, V | + | [[Category: Sellers, V M.]] |
| - | [[Category: Wang, B | + | [[Category: Wang, B C.]] |
| - | [[Category: Wu, C | + | [[Category: Wu, C K.]] |
[[Category: CHD]] | [[Category: CHD]] | ||
[[Category: FES]] | [[Category: FES]] | ||
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[[Category: protoheme ferro-lyase]] | [[Category: protoheme ferro-lyase]] | ||
| - | ''Page seeded by [http:// | + | ''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on Thu Feb 21 13:04:09 2008'' |
Revision as of 11:04, 21 February 2008
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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 and 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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