1pah
From Proteopedia
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| - | [[Image:1pah.gif|left|200px]]<br /><applet load="1pah" size=" | + | [[Image:1pah.gif|left|200px]]<br /><applet load="1pah" size="350" color="white" frame="true" align="right" spinBox="true" |
caption="1pah, resolution 2.0Å" /> | caption="1pah, resolution 2.0Å" /> | ||
'''HUMAN PHENYLALANINE HYDROXYLASE DIMER, RESIDUES 117-424'''<br /> | '''HUMAN PHENYLALANINE HYDROXYLASE DIMER, RESIDUES 117-424'''<br /> | ||
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==About this Structure== | ==About this Structure== | ||
| - | 1PAH 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 FE as [http://en.wikipedia.org/wiki/ligand ligand]. 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]]. Active as [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] Known structural/functional Site: <scene name='pdbsite=NUL:Fe Coordinating Residues'>NUL</scene>. Full crystallographic information is available from [http:// | + | 1PAH 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=FE:'>FE</scene> as [http://en.wikipedia.org/wiki/ligand ligand]. 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]]. Active as [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] Known structural/functional Site: <scene name='pdbsite=NUL:Fe+Coordinating+Residues'>NUL</scene>. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=1PAH OCA]. |
==Reference== | ==Reference== | ||
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[[Category: pku]] | [[Category: pku]] | ||
| - | ''Page seeded by [http:// | + | ''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on Sun Feb 3 09:59:47 2008'' |
Revision as of 07:59, 3 February 2008
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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 with as ligand. The following page contains interesting information on the relation of 1PAH with [Phenylalanine Hydroxylase]. Active as Phenylalanine 4-monooxygenase, with EC number 1.14.16.1 Known structural/functional Site: . 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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