1gr3
From Proteopedia
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| - | [[Image:1gr3. | + | [[Image:1gr3.jpg|left|200px]]<br /><applet load="1gr3" size="450" color="white" frame="true" align="right" spinBox="true" |
| - | <applet load="1gr3" size="450" color="white" frame="true" align="right" spinBox="true" | + | |
caption="1gr3, resolution 2.00Å" /> | caption="1gr3, resolution 2.00Å" /> | ||
'''STRUCTURE OF THE HUMAN COLLAGEN X NC1 TRIMER'''<br /> | '''STRUCTURE OF THE HUMAN COLLAGEN X NC1 TRIMER'''<br /> | ||
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==About this Structure== | ==About this Structure== | ||
| - | 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 CA, NA and CPS as [http://en.wikipedia.org/wiki/ligands ligands]. | + | 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 CA, NA and CPS 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://ispc.weizmann.ac.il/oca-bin/ocashort?id=1GR3 OCA]. |
==Reference== | ==Reference== | ||
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[[Category: extracellular matrix]] | [[Category: extracellular matrix]] | ||
| - | ''Page seeded by [http://ispc.weizmann.ac.il/oca OCA ] on | + | ''Page seeded by [http://ispc.weizmann.ac.il/oca OCA ] on Tue Dec 18 15:32:33 2007'' |
Revision as of 13:22, 18 December 2007
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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 with CA, NA and CPS as ligands. Known structural/functional Site: . 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
Page seeded by OCA on Tue Dec 18 15:32:33 2007
Categories: Homo sapiens | Single protein | Bogin, O. | Hohenester, E. | Kvansakul, M. | Rom, E. | Singer, J. | Yayon, A. | CA | CPS | NA | Collagen | Connective tissue | Extracellular matrix
