1u5m
From Proteopedia
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|ACTIVITY= | |ACTIVITY= | ||
|GENE= COL2A1 ([http://www.ncbi.nlm.nih.gov/Taxonomy/Browser/wwwtax.cgi?mode=Info&srchmode=5&id=9606 Homo sapiens]) | |GENE= COL2A1 ([http://www.ncbi.nlm.nih.gov/Taxonomy/Browser/wwwtax.cgi?mode=Info&srchmode=5&id=9606 Homo sapiens]) | ||
| + | |DOMAIN= | ||
| + | |RELATEDENTRY=[[1fbr|1FBR]] | ||
| + | |RESOURCES=<span class='plainlinks'>[http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=1u5m FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=1u5m OCA], [http://www.ebi.ac.uk/pdbsum/1u5m PDBsum], [http://www.rcsb.org/pdb/explore.do?structureId=1u5m RCSB]</span> | ||
}} | }} | ||
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==Disease== | ==Disease== | ||
| - | Known | + | Known disease associated with this structure: Achondrogenesis-hypochondrogenesis, type II OMIM:[[http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=120140 120140]], Epiphyseal dysplasia, multiple, with myopia and deafness OMIM:[[http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=120140 120140]], Kniest dysplasia OMIM:[[http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=120140 120140]], Osteoarthrosis OMIM:[[http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=120140 120140]], SED congenita OMIM:[[http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=120140 120140]], SED, Namaqualand type OMIM:[[http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=120140 120140]], SMED Strudwick type OMIM:[[http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=120140 120140]], Spondyloperipheral dysplasia OMIM:[[http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=120140 120140]], Stickler syndrome, type I OMIM:[[http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=120140 120140]], Vitreoretinopathy with phalangeal epiphyseal dysplasia ( OMIM:[[http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=120140 120140]] |
==About this Structure== | ==About this Structure== | ||
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[[Category: two sub-domain architecture]] | [[Category: two sub-domain architecture]] | ||
| - | ''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on | + | ''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on Mon Mar 31 00:05:42 2008'' |
Revision as of 21:05, 30 March 2008
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| Gene: | COL2A1 (Homo sapiens) | ||||||
| Related: | 1FBR
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| Resources: | FirstGlance, OCA, PDBsum, RCSB | ||||||
| Coordinates: | save as pdb, mmCIF, xml | ||||||
Structure of a Chordin-like Cysteine-rich Repeat (VWC module) from Collagen IIA
Contents |
Overview
Chordin-like cysteine-rich (CR) repeats (also referred to as von Willebrand factor type C (VWC) modules) have been identified in approximately 200 extracellular matrix proteins. These repeats, named on the basis of amino acid conservation of 10 cysteine residues, have been shown to bind members of the transforming growth factor-beta (TGF-beta) superfamily and are proposed to regulate growth factor signaling. Here we describe the intramolecular disulfide bonding, solution structure, and dynamics of a prototypical chordin-like CR repeat from procollagen IIA (CR(ColIIA)), which has been previously shown to bind TGF-beta1 and bone morphogenetic protein-2. The CR(ColIIA) structure manifests a two sub-domain architecture tethered by a flexible linkage. Initial structures were calculated using RosettaNMR, a de novo prediction method, and final structure calculations were performed using CANDID within CYANA. The N-terminal region contains mainly beta-sheet and the C-terminal region is more irregular with the fold constrained by disulfide bonds. Mobility between the N- and C-terminal sub-domains on a fast timescale was confirmed using NMR relaxation measurements. We speculate that the mobility between the two sub-domains may decrease upon ligand binding. Structure and sequence comparisons have revealed an evolutionary relationship between the N-terminal sub-domain of the CR module and the fibronectin type 1 domain, suggesting that these domains share a common ancestry. Based on the previously reported mapping of fibronectin binding sites for vascular endothelial growth factor to regions containing fibronectin type 1 domains, we discuss the possibility that this structural homology might also have functional relevance.
Disease
Known disease associated with this structure: Achondrogenesis-hypochondrogenesis, type II OMIM:[120140], Epiphyseal dysplasia, multiple, with myopia and deafness OMIM:[120140], Kniest dysplasia OMIM:[120140], Osteoarthrosis OMIM:[120140], SED congenita OMIM:[120140], SED, Namaqualand type OMIM:[120140], SMED Strudwick type OMIM:[120140], Spondyloperipheral dysplasia OMIM:[120140], Stickler syndrome, type I OMIM:[120140], Vitreoretinopathy with phalangeal epiphyseal dysplasia ( OMIM:[120140]
About this Structure
1U5M is a Single protein structure of sequence from Homo sapiens. Full crystallographic information is available from OCA.
Reference
Solution structure and dynamics of a prototypical chordin-like cysteine-rich repeat (von Willebrand Factor type C module) from collagen IIA., O'Leary JM, Hamilton JM, Deane CM, Valeyev NV, Sandell LJ, Downing AK, J Biol Chem. 2004 Dec 17;279(51):53857-66. Epub 2004 Oct 1. PMID:15466413
Page seeded by OCA on Mon Mar 31 00:05:42 2008
