1lm7
From Proteopedia
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|ACTIVITY= | |ACTIVITY= | ||
|GENE= | |GENE= | ||
| + | |DOMAIN= | ||
| + | |RELATEDENTRY= | ||
| + | |RESOURCES=<span class='plainlinks'>[http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=1lm7 FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=1lm7 OCA], [http://www.ebi.ac.uk/pdbsum/1lm7 PDBsum], [http://www.rcsb.org/pdb/explore.do?structureId=1lm7 RCSB]</span> | ||
}} | }} | ||
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==Overview== | ==Overview== | ||
Desmosomes are intercellular junctions in which cadherin cell adhesion molecules are linked to the intermediate filament (IF) system. Desmoplakin is a member of the plakin family of IF-binding proteins. The C-terminal domain of desmoplakin (DPCT) mediates binding to IFs in desmosomes. The DPCT sequence contains three regions, termed A, B and C, consisting of 4.5 copies of a 38-amino acid repeat motif. We demonstrate that these regions form discrete subdomains that bind to IFs and report the crystal structures of domains B and C. In contrast to the elongated structures formed by other kinds of repeat motifs, the plakin repeats form a globular structure with a unique fold. A conserved basic groove found on the domain may represent an IF-binding site. | Desmosomes are intercellular junctions in which cadherin cell adhesion molecules are linked to the intermediate filament (IF) system. Desmoplakin is a member of the plakin family of IF-binding proteins. The C-terminal domain of desmoplakin (DPCT) mediates binding to IFs in desmosomes. The DPCT sequence contains three regions, termed A, B and C, consisting of 4.5 copies of a 38-amino acid repeat motif. We demonstrate that these regions form discrete subdomains that bind to IFs and report the crystal structures of domains B and C. In contrast to the elongated structures formed by other kinds of repeat motifs, the plakin repeats form a globular structure with a unique fold. A conserved basic groove found on the domain may represent an IF-binding site. | ||
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| - | ==Disease== | ||
| - | Known diseases associated with this structure: Arrhythmogenic right ventricular dysplasia 8 OMIM:[[http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=125647 125647]], Dilated cardiomyopathy with woolly hair and keratoderma OMIM:[[http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=125647 125647]], Epidermolysis bullosa, lethal acantholytic OMIM:[[http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=125647 125647]], Keratosis palmoplantaris striata II OMIM:[[http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=125647 125647]], Skin fragility-woolly hair syndrome OMIM:[[http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=125647 125647]] | ||
==About this Structure== | ==About this Structure== | ||
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[[Category: plakin repeat]] | [[Category: plakin repeat]] | ||
| - | ''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 22:04:29 2008'' |
Revision as of 19:04, 30 March 2008
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| , resolution 3.00Å | |||||||
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| Resources: | FirstGlance, OCA, PDBsum, RCSB | ||||||
| Coordinates: | save as pdb, mmCIF, xml | ||||||
Structures of two intermediate filament-binding fragments of desmoplakin reveal a unique repeat motif structure
Overview
Desmosomes are intercellular junctions in which cadherin cell adhesion molecules are linked to the intermediate filament (IF) system. Desmoplakin is a member of the plakin family of IF-binding proteins. The C-terminal domain of desmoplakin (DPCT) mediates binding to IFs in desmosomes. The DPCT sequence contains three regions, termed A, B and C, consisting of 4.5 copies of a 38-amino acid repeat motif. We demonstrate that these regions form discrete subdomains that bind to IFs and report the crystal structures of domains B and C. In contrast to the elongated structures formed by other kinds of repeat motifs, the plakin repeats form a globular structure with a unique fold. A conserved basic groove found on the domain may represent an IF-binding site.
About this Structure
1LM7 is a Single protein structure of sequence from Homo sapiens. Full crystallographic information is available from OCA.
Reference
Structures of two intermediate filament-binding fragments of desmoplakin reveal a unique repeat motif structure., Choi HJ, Park-Snyder S, Pascoe LT, Green KJ, Weis WI, Nat Struct Biol. 2002 Aug;9(8):612-20. PMID:12101406
Page seeded by OCA on Sun Mar 30 22:04:29 2008
