1lm7: Difference between revisions

From Proteopedia
Jump to navigationJump to search
← Older editNewer edit →
OCA (talk | contribs)
Bots, Bureaucrats, journal, Administrators
3,331,613 edits
No edit summary
OCA (talk | contribs)
Bots, Bureaucrats, journal, Administrators
3,331,613 edits
No edit summary
Line 7: Line 7:
|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>
}}
}}


Line 14: Line 17:
==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.
==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==
Line 32: Line 32:
[[Category: plakin repeat]]
[[Category: plakin repeat]]


''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on Thu Mar 20 12:32:31 2008''
''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on Sun Mar 30 22:04:29 2008''
Retrieved from "https://proteopedia.org/1lm7"