1pah
From Proteopedia
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| - | [[Image:1pah.gif|left|200px]] | + | [[Image:1pah.gif|left|200px]] |
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| - | '''HUMAN PHENYLALANINE HYDROXYLASE DIMER, RESIDUES 117-424''' | + | {{Structure |
| + | |PDB= 1pah |SIZE=350|CAPTION= <scene name='initialview01'>1pah</scene>, resolution 2.0Å | ||
| + | |SITE= <scene name='pdbsite=NUL:Fe+Coordinating+Residues'>NUL</scene> | ||
| + | |LIGAND= <scene name='pdbligand=FE:FE (III) ION'>FE</scene> | ||
| + | |ACTIVITY= [http://en.wikipedia.org/wiki/Phenylalanine_4-monooxygenase Phenylalanine 4-monooxygenase], with EC number [http://www.brenda-enzymes.info/php/result_flat.php4?ecno=1.14.16.1 1.14.16.1] | ||
| + | |GENE= | ||
| + | }} | ||
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| + | '''HUMAN PHENYLALANINE HYDROXYLASE DIMER, RESIDUES 117-424''' | ||
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==Overview== | ==Overview== | ||
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==About this Structure== | ==About this Structure== | ||
| - | 1PAH is a [ | + | 1PAH is a [[Single protein]] structure of sequence from [http://en.wikipedia.org/wiki/Homo_sapiens Homo sapiens]. The following page contains interesting information on the relation of 1PAH with [[http://pdb.rcsb.org/pdb/static.do?p=education_discussion/molecule_of_the_month/pdb61_1.html Phenylalanine Hydroxylase]]. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=1PAH OCA]. |
==Reference== | ==Reference== | ||
| - | Crystal structure of the catalytic domain of human phenylalanine hydroxylase reveals the structural basis for phenylketonuria., Erlandsen H, Fusetti F, Martinez A, Hough E, Flatmark T, Stevens RC, Nat Struct Biol. 1997 Dec;4(12):995-1000. PMID:[http:// | + | Crystal structure of the catalytic domain of human phenylalanine hydroxylase reveals the structural basis for phenylketonuria., Erlandsen H, Fusetti F, Martinez A, Hough E, Flatmark T, Stevens RC, Nat Struct Biol. 1997 Dec;4(12):995-1000. PMID:[http://www.ncbi.nlm.nih.gov/pubmed/9406548 9406548] |
[[Category: Homo sapiens]] | [[Category: Homo sapiens]] | ||
[[Category: Phenylalanine 4-monooxygenase]] | [[Category: Phenylalanine 4-monooxygenase]] | ||
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[[Category: pku]] | [[Category: pku]] | ||
| - | ''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on Thu | + | ''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on Thu Mar 20 13:21:58 2008'' |
Revision as of 11:22, 20 March 2008
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| , resolution 2.0Å | |||||||
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| Sites: | |||||||
| Ligands: | |||||||
| Activity: | Phenylalanine 4-monooxygenase, with EC number 1.14.16.1 | ||||||
| Coordinates: | save as pdb, mmCIF, xml | ||||||
HUMAN PHENYLALANINE HYDROXYLASE DIMER, RESIDUES 117-424
Contents |
Overview
The 2.0 A crystal structure of the catalytic domain of human phenylalanine hydroxylase reveals a fold similar to that of tyrosine hydroxylase. It provides the first structural view of where mutations occur and a rationale to explain molecular mechanisms of the enzymatic phenotypes in the autosomal recessive disorder phenylketoneuria.
Disease
Known diseases associated with this structure: Hyperphenylalaninemia, mild OMIM:[261600], Phenylketonuria OMIM:[261600]
About this Structure
1PAH is a Single protein structure of sequence from Homo sapiens. The following page contains interesting information on the relation of 1PAH with [Phenylalanine Hydroxylase]. Full crystallographic information is available from OCA.
Reference
Crystal structure of the catalytic domain of human phenylalanine hydroxylase reveals the structural basis for phenylketonuria., Erlandsen H, Fusetti F, Martinez A, Hough E, Flatmark T, Stevens RC, Nat Struct Biol. 1997 Dec;4(12):995-1000. PMID:9406548
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