2f8v

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|PDB= 2f8v |SIZE=350|CAPTION= <scene name='initialview01'>2f8v</scene>, resolution 2.75&Aring;
|PDB= 2f8v |SIZE=350|CAPTION= <scene name='initialview01'>2f8v</scene>, resolution 2.75&Aring;
|SITE=
|SITE=
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|LIGAND= <scene name='pdbligand=SO4:SULFATE ION'>SO4</scene>
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|LIGAND= <scene name='pdbligand=SO4:SULFATE+ION'>SO4</scene>
|ACTIVITY=
|ACTIVITY=
|GENE= TCAP ([http://www.ncbi.nlm.nih.gov/Taxonomy/Browser/wwwtax.cgi?mode=Info&srchmode=5&id=9606 Homo sapiens])
|GENE= TCAP ([http://www.ncbi.nlm.nih.gov/Taxonomy/Browser/wwwtax.cgi?mode=Info&srchmode=5&id=9606 Homo sapiens])
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|DOMAIN=
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|RELATEDENTRY=[[ya5|YA5]]
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|RESOURCES=<span class='plainlinks'>[http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=2f8v FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=2f8v OCA], [http://www.ebi.ac.uk/pdbsum/2f8v PDBsum], [http://www.rcsb.org/pdb/explore.do?structureId=2f8v RCSB]</span>
}}
}}
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==Disease==
==Disease==
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Known diseases associated with this structure: Cardiomyopathy, dilated, 1G OMIM:[[http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=188840 188840]], Cardiomyopathy, dilated, 1N OMIM:[[http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=604488 604488]], Cardiomyopathy, familial hypertrophic OMIM:[[http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=188840 188840]], Muscular dystrophy, limb-girdle, type 2G OMIM:[[http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=604488 604488]], Muscular dystrophy, limb-girdle, type 2J OMIM:[[http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=188840 188840]], Myopathy, early-onset, with fatal cardiomyopathy OMIM:[[http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=188840 188840]], Myopathy, proximal, with early respiratory muscle involvement OMIM:[[http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=188840 188840]], Tibial muscular dystrophy, tardive OMIM:[[http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=188840 188840]]
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Known disease associated with this structure: Cardiomyopathy, dilated, 1N OMIM:[[http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=604488 604488]], Muscular dystrophy, limb-girdle, type 2G OMIM:[[http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=604488 604488]]
==About this Structure==
==About this Structure==
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[[Category: Wilmanns, M.]]
[[Category: Wilmanns, M.]]
[[Category: Zou, P.]]
[[Category: Zou, P.]]
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[[Category: SO4]]
 
[[Category: sarcomere]]
[[Category: sarcomere]]
[[Category: telethonin]]
[[Category: telethonin]]
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[[Category: z1z2]]
[[Category: z1z2]]
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''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on Thu Mar 20 16:49:27 2008''
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''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on Mon Mar 31 02:59:28 2008''

Revision as of 23:59, 30 March 2008

Image:2f8v.gif


PDB ID 2f8v

Drag the structure with the mouse to rotate
, resolution 2.75Å
Ligands:
Gene: TCAP (Homo sapiens)
Related: YA5


Resources: FirstGlance, OCA, PDBsum, RCSB
Coordinates: save as pdb, mmCIF, xml



Structure of full length telethonin in complex with the N-terminus of titin


Contents

Overview

The Z-disk region defines the lateral boundary of the sarcomere and requires a high level of mechanical strength to provide a stable framework for large filamentous muscle proteins. The level of complexity at this area is reflected by a large number of protein-protein interactions. Recently, we unraveled how the N-terminus of the longest filament component, the giant muscle protein titin, is assembled into an antiparallel (2:1) sandwich complex by the N-terminal titin-binding segment of the Z-disk ligand telethonin/T-cap [Zou, P., Pinotsis, N., Lange, S., Song, Y.H., Popov, A., Mavridis, I., Mayans, O.M., Gautel, M., Wilmanns, M., 2006. Palindromic assembly of the giant muscle protein titin in the sarcomeric Z-disk. Nature 439, 229-233]. In this contribution, we present structural data of a related complex of the titin N-terminus with full-length telethonin. The C-terminus of telethonin remains invisible, suggesting that it does not fold into a defined structure even in the presence of titin. In contrast to the structure with truncated telethonin, a dimer of two titin/telethonin complexes is formed within the crystal environment, potentially indicating the formation of higher oligomers. We further investigated the structure and dynamics of this assembly by small-angle X-ray scattering, circular dichroism, and in vivo complementation data. The data consistently indicate the involvement of the C-terminal part of telethonin into the assembly of two titin/telethonin complexes.

Disease

Known disease associated with this structure: Cardiomyopathy, dilated, 1N OMIM:[604488], Muscular dystrophy, limb-girdle, type 2G OMIM:[604488]

About this Structure

2F8V is a Protein complex structure of sequences from Homo sapiens. Full crystallographic information is available from OCA.

Reference

Evidence for a dimeric assembly of two titin/telethonin complexes induced by the telethonin C-terminus., Pinotsis N, Petoukhov M, Lange S, Svergun D, Zou P, Gautel M, Wilmanns M, J Struct Biol. 2006 Aug;155(2):239-50. Epub 2006 Apr 27. PMID:16713295

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