1lm5 | pdb_00001lm5
Structures of two intermediate filament-binding fragments of desmoplakin reveal a unique repeat motif structure
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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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