1lm5

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'''Structures of two intermediate filament-binding fragments of desmoplakin reveal a unique repeat motif structure'''<br />
'''Structures of two intermediate filament-binding fragments of desmoplakin reveal a unique repeat motif structure'''<br />
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==About this Structure==
==About this Structure==
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1LM5 is a [http://en.wikipedia.org/wiki/Single_protein Single protein] structure of sequence from [http://en.wikipedia.org/wiki/Homo_sapiens Homo sapiens]. Full crystallographic information is available from [http://ispc.weizmann.ac.il/oca-bin/ocashort?id=1LM5 OCA].
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1LM5 is a [http://en.wikipedia.org/wiki/Single_protein Single protein] structure of sequence from [http://en.wikipedia.org/wiki/Homo_sapiens Homo sapiens]. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=1LM5 OCA].
==Reference==
==Reference==
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[[Category: plakin repeat]]
[[Category: plakin repeat]]
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''Page seeded by [http://ispc.weizmann.ac.il/oca OCA ] on Mon Nov 12 18:02:25 2007''
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''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on Fri Feb 15 16:19:11 2008''

Revision as of 14:19, 15 February 2008


1lm5, resolution 1.80Å

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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

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