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1lm5
From Proteopedia
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==Overview== | ==Overview== | ||
| - | Desmosomes are intercellular junctions in which cadherin cell adhesion | + | 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. |
==Disease== | ==Disease== | ||
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[[Category: Homo sapiens]] | [[Category: Homo sapiens]] | ||
[[Category: Single protein]] | [[Category: Single protein]] | ||
| - | [[Category: Choi, H | + | [[Category: Choi, H J.]] |
| - | [[Category: Green, K | + | [[Category: Green, K J.]] |
[[Category: Park-Snyder, S.]] | [[Category: Park-Snyder, S.]] | ||
| - | [[Category: Pascoe, L | + | [[Category: Pascoe, L T.]] |
| - | [[Category: Weis, W | + | [[Category: Weis, W I.]] |
[[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 Thu Feb 21 13:46:13 2008'' |
Revision as of 11:46, 21 February 2008
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Structures of two intermediate filament-binding fragments of desmoplakin reveal a unique repeat motif structure
Contents |
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.
Disease
Known diseases associated with this structure: Arrhythmogenic right ventricular dysplasia 8 OMIM:[125647], Dilated cardiomyopathy with woolly hair and keratoderma OMIM:[125647], Epidermolysis bullosa, lethal acantholytic OMIM:[125647], Keratosis palmoplantaris striata II OMIM:[125647], Skin fragility-woolly hair syndrome OMIM:[125647]
About this Structure
1LM5 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 Thu Feb 21 13:46:13 2008
