1HRK | Lyase | date | Dec 21, 2000 | ||||||||||||
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title | Crystal Structure Of Human Ferrochelatase | ||||||||||||||
authors | C.K.Wu, H.A.Dailey, J.P.Rose, A.Burden, V.M.Sellers, B.-C.Wang | ||||||||||||||
compound | source | ||||||||||||||
Molecule: Ferrochelatase Chain: A, B Fragment: Mature Length Synonym: Protoheme Ferro-Lyase, Heme Synthetase Ec: 4.99.1.1 Engineered: Yes Mutation: Yes |
Organism_scientific: Homo Sapiens Organism_common: Human Expression_system: Escherichia Coli Expression_system_common: Bacteria Expression_system_strain: Jm109 Expression_system_vector_type: Plasmid Expression_system_plasmid: Phdtf20 | ||||||||||||||
symmetry | Space Group: P 21 21 21 | R_factor | 0.202 | ||||||||||||
crystal cell |
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method | X-Ray Diffraction | resolution | 2.00 Å | ||||||||||||
ligand | CHD, FES | enzyme | Ferrochelatase. Ferro-protoporphyrin chelatase. Iron chelatase. Heme synthetase. Heme synthase. Lyase E.C.4.99.1.1 | ||||||||||||
note | 1HRK is a representative structure | ||||||||||||||
similarity | Belongs to the Ferrochelatase family. | ||||||||||||||
subunit | Homodimer. | ||||||||||||||
catalytic activ. | Protoporphyrin + fe(2+) = protoheme + 2 h(+). | ||||||||||||||
pathway | Protoheme biosynthesis; last step. | ||||||||||||||
subcellular loc. | Bound to the mitochondrial inner membrane in eukaryotic cells with its active site on the matrix side of the membrane. | ||||||||||||||
enzyme regulation | The 2fe-2s cluster could act as a no sensor. Inhibited by nitric oxide (no). | ||||||||||||||
genes | BSU10130, HEMF, HEMH (B. subtilis); FECH (H. sapiens); HEM15, MET8, YBR1461, YBR213W, YOR176W (S. cerevisiae) | ||||||||||||||
function | Catalyzes the ferrous insertion into protoporphyrin ix. | ||||||||||||||
Gene Ontology | chain A: GO:0004325, GO:0005739, GO:0006091, GO:0006779, GO:0006783, GO:0008198, GO:0009416, GO:0016829 chain B: GO:0004325, GO:0005739, GO:0006091, GO:0006779, GO:0006783, GO:0008198, GO:0009416, GO:0016829 | ||||||||||||||
disease | Protoporphyria,erythropoietic Protoporphyria,erythropoietic,recessive,with liver failure Depending on the mutation, it can sometimes be recessive. Epp is an dominantly inherited disease of porphyrin metabolism. Defects in fech are the cause of erythropoietic protoporphyria (epp) [mim. 177000]. The clinical manifestations are photosensitivity and hepatobiliary disease. | ||||||||||||||
Primary 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 |
Data retrieval |
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Structure-derived information |
- Domain d1hrka_, region A [Jmol] [rasmolscript] [script source] - Domain d1hrkb_, region B [Jmol] [rasmolscript] [script source] |
Sequence-derived information |
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