2f8v: Difference between revisions
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'''Structure of full length telethonin in complex with the N-terminus of titin''' | {{Structure | ||
|PDB= 2f8v |SIZE=350|CAPTION= <scene name='initialview01'>2f8v</scene>, resolution 2.75Å | |||
|SITE= | |||
|LIGAND= <scene name='pdbligand=SO4:SULFATE ION'>SO4</scene> | |||
|ACTIVITY= | |||
|GENE= TCAP ([http://www.ncbi.nlm.nih.gov/Taxonomy/Browser/wwwtax.cgi?mode=Info&srchmode=5&id=9606 Homo sapiens]) | |||
}} | |||
'''Structure of full length telethonin in complex with the N-terminus of titin''' | |||
==Overview== | ==Overview== | ||
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==About this Structure== | ==About this Structure== | ||
2F8V is a [ | 2F8V is a [[Protein complex]] structure of sequences 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=2F8V OCA]. | ||
==Reference== | ==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:[http:// | 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:[http://www.ncbi.nlm.nih.gov/pubmed/16713295 16713295] | ||
[[Category: Homo sapiens]] | [[Category: Homo sapiens]] | ||
[[Category: Protein complex]] | [[Category: Protein complex]] | ||
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[[Category: z1z2]] | [[Category: z1z2]] | ||
''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on Thu | ''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on Thu Mar 20 16:49:27 2008'' | ||
Revision as of 14:49, 20 March 2008
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| 2f8v, resolution 2.75Å | |||||||||||||
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| Ligands: | SO4 | ||||||||||||
| Gene: | TCAP (Homo sapiens) | ||||||||||||
| Coordinates: | save as pdb, mmCIF, xml | ||||||||||||
Structure of full length telethonin in complex with the N-terminus of titin
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 diseases associated with this structure: Cardiomyopathy, dilated, 1G OMIM:[188840], Cardiomyopathy, dilated, 1N OMIM:[604488], Cardiomyopathy, familial hypertrophic OMIM:[188840], Muscular dystrophy, limb-girdle, type 2G OMIM:[604488], Muscular dystrophy, limb-girdle, type 2J OMIM:[188840], Myopathy, early-onset, with fatal cardiomyopathy OMIM:[188840], Myopathy, proximal, with early respiratory muscle involvement OMIM:[188840], Tibial muscular dystrophy, tardive OMIM:[188840]
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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