1gr3

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[[Image:1gr3.jpg|left|200px]]<br /><applet load="1gr3" size="350" color="white" frame="true" align="right" spinBox="true"
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[[Image:1gr3.jpg|left|200px]]
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caption="1gr3, resolution 2.00&Aring;" />
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'''STRUCTURE OF THE HUMAN COLLAGEN X NC1 TRIMER'''<br />
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{{Structure
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|PDB= 1gr3 |SIZE=350|CAPTION= <scene name='initialview01'>1gr3</scene>, resolution 2.00&Aring;
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|SITE= <scene name='pdbsite=CPS:Cps+Binding+Site+For+Chain+A'>CPS</scene>
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|LIGAND= <scene name='pdbligand=CA:CALCIUM+ION'>CA</scene>, <scene name='pdbligand=NA:SODIUM+ION'>NA</scene> and <scene name='pdbligand=CPS:3-[(3-CHOLAMIDOPROPYL)DIMETHYLAMMONIO]-1-PROPANESULFONATE'>CPS</scene>
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|ACTIVITY=
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|GENE=
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}}
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'''STRUCTURE OF THE HUMAN COLLAGEN X NC1 TRIMER'''
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==Overview==
==Overview==
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==About this Structure==
==About this Structure==
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1GR3 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=CA:'>CA</scene>, <scene name='pdbligand=NA:'>NA</scene> and <scene name='pdbligand=CPS:'>CPS</scene> as [http://en.wikipedia.org/wiki/ligands ligands]. Known structural/functional Site: <scene name='pdbsite=CPS:Cps+Binding+Site+For+Chain+A'>CPS</scene>. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=1GR3 OCA].
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1GR3 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=1GR3 OCA].
==Reference==
==Reference==
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Insight into Schmid metaphyseal chondrodysplasia from the crystal structure of the collagen X NC1 domain trimer., Bogin O, Kvansakul M, Rom E, Singer J, Yayon A, Hohenester E, Structure. 2002 Feb;10(2):165-73. PMID:[http://ispc.weizmann.ac.il//pmbin/getpm?pmid=11839302 11839302]
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Insight into Schmid metaphyseal chondrodysplasia from the crystal structure of the collagen X NC1 domain trimer., Bogin O, Kvansakul M, Rom E, Singer J, Yayon A, Hohenester E, Structure. 2002 Feb;10(2):165-73. PMID:[http://www.ncbi.nlm.nih.gov/pubmed/11839302 11839302]
[[Category: Homo sapiens]]
[[Category: Homo sapiens]]
[[Category: Single protein]]
[[Category: Single protein]]
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[[Category: extracellular matrix]]
[[Category: extracellular matrix]]
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''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on Thu Feb 21 12:53:19 2008''
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''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on Thu Mar 20 11:26:55 2008''

Revision as of 09:26, 20 March 2008


PDB ID 1gr3

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, resolution 2.00Å
Sites:
Ligands: , and
Coordinates: save as pdb, mmCIF, xml



STRUCTURE OF THE HUMAN COLLAGEN X NC1 TRIMER


Contents

Overview

Collagen X is expressed specifically in the growth plate of long bones. Its C1q-like C-terminal NC1 domain forms a stable homotrimer and is crucial for collagen X assembly. Mutations in the NC1 domain cause Schmid metaphyseal chondrodysplasia (SMCD). The crystal structure at 2.0 A resolution of the human collagen X NC1 domain reveals an intimate trimeric assembly strengthened by a buried cluster of calcium ions. Three strips of exposed aromatic residues on the surface of NC1 trimer are likely to be involved in the supramolecular assembly of collagen X. Most internal SMCD mutations probably prevent protein folding, whereas mutations of surface residues may affect the collagen X suprastructure in a dominant-negative manner.

Disease

Known diseases associated with this structure: Metaphyseal chondrodysplasia, Schmid type OMIM:[120110], Spondylometaphyseal dysplasia, Japanese type OMIM:[120110]

About this Structure

1GR3 is a Single protein structure of sequence from Homo sapiens. Full crystallographic information is available from OCA.

Reference

Insight into Schmid metaphyseal chondrodysplasia from the crystal structure of the collagen X NC1 domain trimer., Bogin O, Kvansakul M, Rom E, Singer J, Yayon A, Hohenester E, Structure. 2002 Feb;10(2):165-73. PMID:11839302

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