1gr3
From Proteopedia
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|PDB= 1gr3 |SIZE=350|CAPTION= <scene name='initialview01'>1gr3</scene>, resolution 2.00Å | |PDB= 1gr3 |SIZE=350|CAPTION= <scene name='initialview01'>1gr3</scene>, resolution 2.00Å | ||
|SITE= <scene name='pdbsite=CPS:Cps+Binding+Site+For+Chain+A'>CPS</scene> | |SITE= <scene name='pdbsite=CPS:Cps+Binding+Site+For+Chain+A'>CPS</scene> | ||
| - | |LIGAND= <scene name='pdbligand=CA:CALCIUM+ION'>CA</scene>, | + | |LIGAND= <scene name='pdbligand=CA:CALCIUM+ION'>CA</scene>, <scene name='pdbligand=CPS:3-[(3-CHOLAMIDOPROPYL)DIMETHYLAMMONIO]-1-PROPANESULFONATE'>CPS</scene>, <scene name='pdbligand=NA:SODIUM+ION'>NA</scene> |
|ACTIVITY= | |ACTIVITY= | ||
|GENE= | |GENE= | ||
| + | |DOMAIN= | ||
| + | |RELATEDENTRY= | ||
| + | |RESOURCES=<span class='plainlinks'>[http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=1gr3 FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=1gr3 OCA], [http://www.ebi.ac.uk/pdbsum/1gr3 PDBsum], [http://www.rcsb.org/pdb/explore.do?structureId=1gr3 RCSB]</span> | ||
}} | }} | ||
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==Overview== | ==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. | 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. | ||
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| - | ==Disease== | ||
| - | Known diseases associated with this structure: Metaphyseal chondrodysplasia, Schmid type OMIM:[[http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=120110 120110]], Spondylometaphyseal dysplasia, Japanese type OMIM:[[http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=120110 120110]] | ||
==About this Structure== | ==About this Structure== | ||
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[[Category: Singer, J.]] | [[Category: Singer, J.]] | ||
[[Category: Yayon, A.]] | [[Category: Yayon, A.]] | ||
| - | [[Category: CA]] | ||
| - | [[Category: CPS]] | ||
| - | [[Category: NA]] | ||
[[Category: collagen]] | [[Category: collagen]] | ||
[[Category: connective tissue]] | [[Category: connective tissue]] | ||
[[Category: extracellular matrix]] | [[Category: extracellular matrix]] | ||
| - | ''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on | + | ''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on Sun Mar 30 20:49:39 2008'' |
Revision as of 17:49, 30 March 2008
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| , resolution 2.00Å | |||||||
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| Sites: | |||||||
| Ligands: | , , | ||||||
| Resources: | FirstGlance, OCA, PDBsum, RCSB | ||||||
| Coordinates: | save as pdb, mmCIF, xml | ||||||
STRUCTURE OF THE HUMAN COLLAGEN X NC1 TRIMER
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.
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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