2pz5
From Proteopedia
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| - | [[Image:2pz5.jpg|left|200px]] | + | [[Image:2pz5.jpg|left|200px]] |
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| - | '''Crystal Strucure of FGF Receptor 2 (FGFR2) Kinase Domain Harboring the Pathogenic N549T Mutation Responsible for Pfeiffer Syndrome''' | + | {{Structure |
| + | |PDB= 2pz5 |SIZE=350|CAPTION= <scene name='initialview01'>2pz5</scene>, resolution 2.4Å | ||
| + | |SITE= | ||
| + | |LIGAND= <scene name='pdbligand=SO4:SULFATE+ION'>SO4</scene>, <scene name='pdbligand=MG:MAGNESIUM+ION'>MG</scene> and <scene name='pdbligand=ABG:ADENOSINE 5'-[BETA,GAMMA-METHYLENE]TRIPHOSPHATE'>ABG</scene> | ||
| + | |ACTIVITY= [http://en.wikipedia.org/wiki/Receptor_protein-tyrosine_kinase Receptor protein-tyrosine kinase], with EC number [http://www.brenda-enzymes.info/php/result_flat.php4?ecno=2.7.10.1 2.7.10.1] | ||
| + | |GENE= FGFR2, BEK, KSAM ([http://www.ncbi.nlm.nih.gov/Taxonomy/Browser/wwwtax.cgi?mode=Info&srchmode=5&id=9606 Homo sapiens]) | ||
| + | }} | ||
| + | |||
| + | '''Crystal Strucure of FGF Receptor 2 (FGFR2) Kinase Domain Harboring the Pathogenic N549T Mutation Responsible for Pfeiffer Syndrome''' | ||
| + | |||
==Overview== | ==Overview== | ||
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==About this Structure== | ==About this Structure== | ||
| - | 2PZ5 is a [ | + | 2PZ5 is a [[Single protein]] structure of sequence from [http://en.wikipedia.org/wiki/Homo_sapiens Homo sapiens]. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=2PZ5 OCA]. |
==Reference== | ==Reference== | ||
| - | A molecular brake in the kinase hinge region regulates the activity of receptor tyrosine kinases., Chen H, Ma J, Li W, Eliseenkova AV, Xu C, Neubert TA, Miller WT, Mohammadi M, Mol Cell. 2007 Sep 7;27(5):717-30. PMID:[http:// | + | A molecular brake in the kinase hinge region regulates the activity of receptor tyrosine kinases., Chen H, Ma J, Li W, Eliseenkova AV, Xu C, Neubert TA, Miller WT, Mohammadi M, Mol Cell. 2007 Sep 7;27(5):717-30. PMID:[http://www.ncbi.nlm.nih.gov/pubmed/17803937 17803937] |
[[Category: Homo sapiens]] | [[Category: Homo sapiens]] | ||
[[Category: Receptor protein-tyrosine kinase]] | [[Category: Receptor protein-tyrosine kinase]] | ||
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[[Category: MG]] | [[Category: MG]] | ||
[[Category: SO4]] | [[Category: SO4]] | ||
| - | [[Category: kinase domain fold consisting of n- and c- | + | [[Category: kinase domain fold consisting of n- and c-lobe]] |
[[Category: transferase]] | [[Category: transferase]] | ||
| - | ''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 18:19:46 2008'' |
Revision as of 16:19, 20 March 2008
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| , resolution 2.4Å | |||||||
|---|---|---|---|---|---|---|---|
| Ligands: | , and | ||||||
| Gene: | FGFR2, BEK, KSAM (Homo sapiens) | ||||||
| Activity: | Receptor protein-tyrosine kinase, with EC number 2.7.10.1 | ||||||
| Coordinates: | save as pdb, mmCIF, xml | ||||||
Crystal Strucure of FGF Receptor 2 (FGFR2) Kinase Domain Harboring the Pathogenic N549T Mutation Responsible for Pfeiffer Syndrome
Contents |
Overview
Activating mutations in the tyrosine kinase domain of receptor tyrosine kinases (RTKs) cause cancer and skeletal disorders. Comparison of the crystal structures of unphosphorylated and phosphorylated wild-type FGFR2 kinase domains with those of seven unphosphorylated pathogenic mutants reveals an autoinhibitory "molecular brake" mediated by a triad of residues in the kinase hinge region of all FGFRs. Structural analysis shows that many other RTKs, including PDGFRs, VEGFRs, KIT, CSF1R, FLT3, TEK, and TIE, are also subject to regulation by this brake. Pathogenic mutations activate FGFRs and other RTKs by disengaging the brake either directly or indirectly.
Disease
Known diseases associated with this structure: Antley-Bixler syndrome, 207410 ( OMIM:[176943], Apert syndrome OMIM:[176943], Beare-Stevenson cutis gyrata syndrome OMIM:[176943], Craniofacial-skeletal-dermatologic dysplasia OMIM:[176943], Craniosynostosis, nonspecific OMIM:[176943], Crouzon syndrome OMIM:[176943], Gastric cancer, somatic OMIM:[176943], Jackson-Weiss syndrome OMIM:[176943], Pfeiffer syndrome OMIM:[176943], Saethre-Chotzen syndrome OMIM:[176943]
About this Structure
2PZ5 is a Single protein structure of sequence from Homo sapiens. Full crystallographic information is available from OCA.
Reference
A molecular brake in the kinase hinge region regulates the activity of receptor tyrosine kinases., Chen H, Ma J, Li W, Eliseenkova AV, Xu C, Neubert TA, Miller WT, Mohammadi M, Mol Cell. 2007 Sep 7;27(5):717-30. PMID:17803937
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