1ii4

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{{STRUCTURE_1ii4| PDB=1ii4 | SCENE= }}
{{STRUCTURE_1ii4| PDB=1ii4 | SCENE= }}
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'''CRYSTAL STRUCTURE OF SER252TRP APERT MUTANT FGF RECEPTOR 2 (FGFR2) IN COMPLEX WITH FGF2'''
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===CRYSTAL STRUCTURE OF SER252TRP APERT MUTANT FGF RECEPTOR 2 (FGFR2) IN COMPLEX WITH FGF2===
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==Overview==
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Apert syndrome (AS) is characterized by craniosynostosis (premature fusion of cranial sutures) and severe syndactyly of the hands and feet. Two activating mutations, Ser-252 --&gt; Trp and Pro-253 --&gt; Arg, in fibroblast growth factor receptor 2 (FGFR2) account for nearly all known cases of AS. To elucidate the mechanism by which these substitutions cause AS, we determined the crystal structures of these two FGFR2 mutants in complex with fibroblast growth factor 2 (FGF2). These structures demonstrate that both mutations introduce additional interactions between FGFR2 and FGF2, thereby augmenting FGFR2-FGF2 affinity. Moreover, based on these structures and sequence alignment of the FGF family, we propose that the Pro-253 --&gt; Arg mutation will indiscriminately increase the affinity of FGFR2 toward any FGF. In contrast, the Ser-252 --&gt; Trp mutation will selectively enhance the affinity of FGFR2 toward a limited subset of FGFs. These predictions are consistent with previous biochemical data describing the effects of AS mutations on FGF binding. Alterations in FGFR2 ligand affinity and specificity may allow inappropriate autocrine or paracrine activation of FGFR2. Furthermore, the distinct gain-of-function interactions observed in each crystal structure provide a model to explain the phenotypic variability among AS patients.
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(as it appears on PubMed at http://www.pubmed.gov), where 11390973 is the PubMed ID number.
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{{ABSTRACT_PUBMED_11390973}}
==About this Structure==
==About this Structure==
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[[Category: B-trefoil]]
[[Category: B-trefoil]]
[[Category: Immunoglobulin like domain]]
[[Category: Immunoglobulin like domain]]
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''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on Fri May 2 20:01:40 2008''
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''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on Tue Jul 1 12:14:40 2008''

Revision as of 09:14, 1 July 2008

Image:1ii4.png

Template:STRUCTURE 1ii4

CRYSTAL STRUCTURE OF SER252TRP APERT MUTANT FGF RECEPTOR 2 (FGFR2) IN COMPLEX WITH FGF2

Template:ABSTRACT PUBMED 11390973

About this Structure

1II4 is a Protein complex structure of sequences from Homo sapiens. Full crystallographic information is available from OCA.

Reference

Structural basis for fibroblast growth factor receptor 2 activation in Apert syndrome., Ibrahimi OA, Eliseenkova AV, Plotnikov AN, Yu K, Ornitz DM, Mohammadi M, Proc Natl Acad Sci U S A. 2001 Jun 19;98(13):7182-7. Epub 2001 Jun 5. PMID:11390973

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