1q7d

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{{STRUCTURE_1q7d| PDB=1q7d | SCENE= }}
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==Structure of the integrin alpha2beta1 binding collagen peptide==
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===Structure of the integrin alpha2beta1 binding collagen peptide===
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<StructureSection load='1q7d' size='340' side='right' caption='[[1q7d]], [[Resolution|resolution]] 1.80&Aring;' scene=''>
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{{ABSTRACT_PUBMED_14698296}}
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== Structural highlights ==
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<table><tr><td colspan='2'>[[1q7d]] is a 3 chain structure. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=1Q7D OCA]. For a <b>guided tour on the structure components</b> use [http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=1Q7D FirstGlance]. <br>
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</td></tr><tr id='NonStdRes'><td class="sblockLbl"><b>[[Non-Standard_Residue|NonStd Res:]]</b></td><td class="sblockDat"><scene name='pdbligand=ACE:ACETYL+GROUP'>ACE</scene>, <scene name='pdbligand=HYP:4-HYDROXYPROLINE'>HYP</scene>, <scene name='pdbligand=NH2:AMINO+GROUP'>NH2</scene></td></tr>
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<tr id='related'><td class="sblockLbl"><b>[[Related_structure|Related:]]</b></td><td class="sblockDat">[[1dzi|1dzi]]</td></tr>
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<tr id='resources'><td class="sblockLbl"><b>Resources:</b></td><td class="sblockDat"><span class='plainlinks'>[http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=1q7d FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=1q7d OCA], [http://www.rcsb.org/pdb/explore.do?structureId=1q7d RCSB], [http://www.ebi.ac.uk/pdbsum/1q7d PDBsum]</span></td></tr>
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</table>
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== Disease ==
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[[http://www.uniprot.org/uniprot/CO1A1_HUMAN CO1A1_HUMAN]] Defects in COL1A1 are the cause of Caffey disease (CAFFD) [MIM:[http://omim.org/entry/114000 114000]]; also known as infantile cortical hyperostosis. Caffey disease is characterized by an infantile episode of massive subperiosteal new bone formation that typically involves the diaphyses of the long bones, mandible, and clavicles. The involved bones may also appear inflamed, with painful swelling and systemic fever often accompanying the illness. The bone changes usually begin before 5 months of age and resolve before 2 years of age.<ref>PMID:8988177</ref> <ref>PMID:12660034</ref> <ref>PMID:15864348</ref> Defects in COL1A1 are a cause of Ehlers-Danlos syndrome type 1 (EDS1) [MIM:[http://omim.org/entry/130000 130000]]; also known as Ehlers-Danlos syndrome gravis. EDS is a connective tissue disorder characterized by hyperextensible skin, atrophic cutaneous scars due to tissue fragility and joint hyperlaxity. EDS1 is the severe form of classic Ehlers-Danlos syndrome.<ref>PMID:8988177</ref> <ref>PMID:12660034</ref> <ref>PMID:10739762</ref> <ref>PMID:17211858</ref> Defects in COL1A1 are the cause of Ehlers-Danlos syndrome type 7A (EDS7A) [MIM:[http://omim.org/entry/130060 130060]]; also known as autosomal dominant Ehlers-Danlos syndrome type VII. EDS is a connective tissue disorder characterized by hyperextensible skin, atrophic cutaneous scars due to tissue fragility and joint hyperlaxity. EDS7A is marked by bilateral congenital hip dislocation, hyperlaxity of the joints, and recurrent partial dislocations.<ref>PMID:8988177</ref> <ref>PMID:12660034</ref> Defects in COL1A1 are a cause of osteogenesis imperfecta type 1 (OI1) [MIM:[http://omim.org/entry/166200 166200]]. A dominantly inherited connective tissue disorder characterized by bone fragility and blue sclerae. Osteogenesis imperfecta type 1 is non-deforming with normal height or mild short stature, and no dentinogenesis imperfecta.<ref>PMID:8988177</ref> <ref>PMID:12660034</ref> <ref>PMID:3244312</ref> <ref>PMID:2794057</ref> <ref>PMID:1718984</ref> <ref>PMID:1634225</ref> <ref>PMID:1737847</ref> <ref>PMID:8223589</ref> <ref>PMID:16705691</ref> <ref>PMID:16786509</ref> <ref>PMID:16638323</ref> <ref>PMID:17875077</ref> <ref>PMID:18670065</ref> Defects in COL1A1 are a cause of osteogenesis imperfecta type 2 (OI2) [MIM:[http://omim.org/entry/166210 166210]]; also known as osteogenesis imperfecta congenita. A connective tissue disorder characterized by bone fragility, with many perinatal fractures, severe bowing of long bones, undermineralization, and death in the perinatal period due to respiratory insufficiency. Defects in COL1A1 are a cause of osteogenesis imperfecta type 3 (OI3) [MIM:[http://omim.org/entry/259420 259420]]. A connective tissue disorder characterized by progressively deforming bones, very short stature, a triangular face, severe scoliosis, grayish sclera, and dentinogenesis imperfecta. Defects in COL1A1 are a cause of osteogenesis imperfecta type 4 (OI4) [MIM:[http://omim.org/entry/166220 166220]]; also known as osteogenesis imperfecta with normal sclerae. A connective tissue disorder characterized by moderately short stature, mild to moderate scoliosis, grayish or white sclera and dentinogenesis imperfecta. Genetic variations in COL1A1 are a cause of susceptibility to osteoporosis (OSTEOP) [MIM:[http://omim.org/entry/166710 166710]]; also known as involutional or senile osteoporosis or postmenopausal osteoporosis. Osteoporosis is characterized by reduced bone mass, disruption of bone microarchitecture without alteration in the composition of bone. Osteoporotic bones are more at risk of fracture.<ref>PMID:8988177</ref> <ref>PMID:12660034</ref> <ref>PMID:8841196</ref> <ref>PMID:9535665</ref> Note=A chromosomal aberration involving COL1A1 is found in dermatofibrosarcoma protuberans. Translocation t(17;22)(q22;q13) with PDGF.<ref>PMID:8988177</ref> <ref>PMID:12660034</ref>
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== Function ==
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[[http://www.uniprot.org/uniprot/CO1A1_HUMAN CO1A1_HUMAN]] Type I collagen is a member of group I collagen (fibrillar forming collagen).
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<div style="background-color:#fffaf0;">
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== Publication Abstract from PubMed ==
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We have determined the 1.8A crystal structure of a triple helical integrin-binding collagen peptide (IBP) with sequence (Gly-Pro-Hyp)(2)-Gly-Phe-Hyp-Gly-Glu-Arg-(Gly-Pro-Hyp)(3). The central GFOGER hexapeptide is recognised specifically by the integrins alpha2beta1, alpha1beta1, alpha10beta1 and alpha11beta1. These integrin/collagen interactions are implicated in a number of key physiological processes including cell adhesion, cell growth and differentiation, and pathological states such as thrombosis and tumour metastasis. Comparison of the IBP structure with the previously determined structure of an identical collagen peptide in complex with the integrin alpha2-I domain (IBP(c)) allows the first detailed examination of collagen in a bound and an unbound state. The IBP structure shows a direct and a water-mediated electrostatic interaction between Glu and Arg side-chains from adjacent strands, but no intra-strand interactions. The interactions between IBP Glu and Arg side-chains are disrupted upon integrin binding. A comparison of IBP and IBP(c) main-chain conformation reveals the flexible nature of the triple helix backbone in the imino-poor GFOGER region. This flexibility could be important to the integrin-collagen interaction and provides a possible explanation for the unique orientation of the three GFOGER strands observed in the integrin-IBP(c) complex crystal structure.
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==Disease==
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Structure of the integrin alpha2beta1-binding collagen peptide.,Emsley J, Knight CG, Farndale RW, Barnes MJ J Mol Biol. 2004 Jan 23;335(4):1019-28. PMID:14698296<ref>PMID:14698296</ref>
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[[http://www.uniprot.org/uniprot/CO1A1_HUMAN CO1A1_HUMAN]] Defects in COL1A1 are the cause of Caffey disease (CAFFD) [MIM:[http://omim.org/entry/114000 114000]]; also known as infantile cortical hyperostosis. Caffey disease is characterized by an infantile episode of massive subperiosteal new bone formation that typically involves the diaphyses of the long bones, mandible, and clavicles. The involved bones may also appear inflamed, with painful swelling and systemic fever often accompanying the illness. The bone changes usually begin before 5 months of age and resolve before 2 years of age.<ref>PMID:8988177</ref><ref>PMID:12660034</ref><ref>PMID:15864348</ref> Defects in COL1A1 are a cause of Ehlers-Danlos syndrome type 1 (EDS1) [MIM:[http://omim.org/entry/130000 130000]]; also known as Ehlers-Danlos syndrome gravis. EDS is a connective tissue disorder characterized by hyperextensible skin, atrophic cutaneous scars due to tissue fragility and joint hyperlaxity. EDS1 is the severe form of classic Ehlers-Danlos syndrome.<ref>PMID:8988177</ref><ref>PMID:12660034</ref><ref>PMID:10739762</ref><ref>PMID:17211858</ref> Defects in COL1A1 are the cause of Ehlers-Danlos syndrome type 7A (EDS7A) [MIM:[http://omim.org/entry/130060 130060]]; also known as autosomal dominant Ehlers-Danlos syndrome type VII. EDS is a connective tissue disorder characterized by hyperextensible skin, atrophic cutaneous scars due to tissue fragility and joint hyperlaxity. EDS7A is marked by bilateral congenital hip dislocation, hyperlaxity of the joints, and recurrent partial dislocations.<ref>PMID:8988177</ref><ref>PMID:12660034</ref> Defects in COL1A1 are a cause of osteogenesis imperfecta type 1 (OI1) [MIM:[http://omim.org/entry/166200 166200]]. A dominantly inherited connective tissue disorder characterized by bone fragility and blue sclerae. Osteogenesis imperfecta type 1 is non-deforming with normal height or mild short stature, and no dentinogenesis imperfecta.<ref>PMID:8988177</ref><ref>PMID:12660034</ref><ref>PMID:3244312</ref><ref>PMID:2794057</ref><ref>PMID:1718984</ref><ref>PMID:1634225</ref><ref>PMID:1737847</ref><ref>PMID:8223589</ref><ref>PMID:16705691</ref><ref>PMID:16786509</ref><ref>PMID:16638323</ref><ref>PMID:17875077</ref><ref>PMID:18670065</ref> Defects in COL1A1 are a cause of osteogenesis imperfecta type 2 (OI2) [MIM:[http://omim.org/entry/166210 166210]]; also known as osteogenesis imperfecta congenita. A connective tissue disorder characterized by bone fragility, with many perinatal fractures, severe bowing of long bones, undermineralization, and death in the perinatal period due to respiratory insufficiency. Defects in COL1A1 are a cause of osteogenesis imperfecta type 3 (OI3) [MIM:[http://omim.org/entry/259420 259420]]. A connective tissue disorder characterized by progressively deforming bones, very short stature, a triangular face, severe scoliosis, grayish sclera, and dentinogenesis imperfecta. Defects in COL1A1 are a cause of osteogenesis imperfecta type 4 (OI4) [MIM:[http://omim.org/entry/166220 166220]]; also known as osteogenesis imperfecta with normal sclerae. A connective tissue disorder characterized by moderately short stature, mild to moderate scoliosis, grayish or white sclera and dentinogenesis imperfecta. Genetic variations in COL1A1 are a cause of susceptibility to osteoporosis (OSTEOP) [MIM:[http://omim.org/entry/166710 166710]]; also known as involutional or senile osteoporosis or postmenopausal osteoporosis. Osteoporosis is characterized by reduced bone mass, disruption of bone microarchitecture without alteration in the composition of bone. Osteoporotic bones are more at risk of fracture.<ref>PMID:8988177</ref><ref>PMID:12660034</ref><ref>PMID:8841196</ref><ref>PMID:9535665</ref> Note=A chromosomal aberration involving COL1A1 is found in dermatofibrosarcoma protuberans. Translocation t(17;22)(q22;q13) with PDGF.<ref>PMID:8988177</ref><ref>PMID:12660034</ref>
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==Function==
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From MEDLINE&reg;/PubMed&reg;, a database of the U.S. National Library of Medicine.<br>
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[[http://www.uniprot.org/uniprot/CO1A1_HUMAN CO1A1_HUMAN]] Type I collagen is a member of group I collagen (fibrillar forming collagen).
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</div>
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==About this Structure==
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[[1q7d]] is a 3 chain structure. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=1Q7D OCA].
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==See Also==
==See Also==
*[[Collagen|Collagen]]
*[[Collagen|Collagen]]
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== References ==
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==Reference==
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<references/>
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<ref group="xtra">PMID:014698296</ref><references group="xtra"/><references/>
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__TOC__
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[[Category: Barnes, M J.]]
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</StructureSection>
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[[Category: Emsley, J.]]
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[[Category: Barnes, M J]]
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[[Category: Farndale, R W.]]
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[[Category: Emsley, J]]
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[[Category: Knight, C G.]]
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[[Category: Farndale, R W]]
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[[Category: Knight, C G]]
[[Category: Collagen]]
[[Category: Collagen]]
[[Category: Contractile protein]]
[[Category: Contractile protein]]
[[Category: Integrin]]
[[Category: Integrin]]
[[Category: Triplehelix]]
[[Category: Triplehelix]]

Revision as of 12:48, 18 December 2014

Structure of the integrin alpha2beta1 binding collagen peptide

1q7d, resolution 1.80Å

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