Group:MUZIC:Telethonin

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It is a small protein of 19kDa, 167 amino acids. It is predominantly expressed in striated muscle. It is a structural protein of the muscle; it is associated to the Z-disc in the sarcomere. It acts as multifunctional protein linking titin and other proteins implicated in sarcomere structure and signalling pathways.
It is a small protein of 19kDa, 167 amino acids. It is predominantly expressed in striated muscle. It is a structural protein of the muscle; it is associated to the Z-disc in the sarcomere. It acts as multifunctional protein linking titin and other proteins implicated in sarcomere structure and signalling pathways.
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== Gene regulation and expression ==
== Gene regulation and expression ==
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At the transcriptional level it is thought that there is only one isoform of Tcap, and it is one of the most abundant transcripts in skeletal muscle <ref>PMID:9350988</ref>. It does not have different levels of expression in different types of skeletal muscle; levels of expression of ''Tcap'' are lower in neonatal compared to adult striated muscle. The transcript is accumulated in a linear pattern similar to that of the myosin heavy chain <ref>PMID: 10208846</ref>. In these same studies it was reported that denervation leads to decrease in the expression of Tcap, suggesting that locomotor activity is a potential regulator of its maintenance.
At the transcriptional level it is thought that there is only one isoform of Tcap, and it is one of the most abundant transcripts in skeletal muscle <ref>PMID:9350988</ref>. It does not have different levels of expression in different types of skeletal muscle; levels of expression of ''Tcap'' are lower in neonatal compared to adult striated muscle. The transcript is accumulated in a linear pattern similar to that of the myosin heavy chain <ref>PMID: 10208846</ref>. In these same studies it was reported that denervation leads to decrease in the expression of Tcap, suggesting that locomotor activity is a potential regulator of its maintenance.
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Telethonin protein is found mostly in skeletal and cardiac muscle. It is one of the major components of the sarcomere, it is localized to the Z-disc. It was also reported a nuclear localization.<ref>PMID:12379311 </ref> <ref>PMID:16678796 </ref>
 
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Telethonin protein is found mostly in skeletal and cardiac muscle. It is one of the major components of the sarcomere, it is localized to the Z-disc. It was also reported a nuclear localization.<ref>PMID:12379311 </ref> <ref>PMID:16678796 </ref>
Studies on telethonin structure by Zou et al. <ref>PMID:16407954</ref> report that it is made up of <scene name='User:Marcia_Ivonne_Pena_Paz/workbench/Telethonin/Telethonin_nter_cter/1'>five stranded antiparallel β-sheets extended by two wing-shaped β-hairpin motifs (A-B, C-D). These two motifs are related by an approximate two-fold symmetry, which generates an almost perfect palindromic arrangement.</scene> (N-terminal in blue and C-ter in orange)
Studies on telethonin structure by Zou et al. <ref>PMID:16407954</ref> report that it is made up of <scene name='User:Marcia_Ivonne_Pena_Paz/workbench/Telethonin/Telethonin_nter_cter/1'>five stranded antiparallel β-sheets extended by two wing-shaped β-hairpin motifs (A-B, C-D). These two motifs are related by an approximate two-fold symmetry, which generates an almost perfect palindromic arrangement.</scene> (N-terminal in blue and C-ter in orange)
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There is an interaction with MDM2 N-terminal. MDM2 is capable of redirecting telethonin to the nucleus. Telethonin is inhibited by MDM2 in a dose dependent manner. In cells MDM2 is involved in the regulation of proteasomal turnover of telethonin. <ref>PMID:16678796 </ref>
There is an interaction with MDM2 N-terminal. MDM2 is capable of redirecting telethonin to the nucleus. Telethonin is inhibited by MDM2 in a dose dependent manner. In cells MDM2 is involved in the regulation of proteasomal turnover of telethonin. <ref>PMID:16678796 </ref>
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== Pathologies associated with telethonin ==
== Pathologies associated with telethonin ==
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Revision as of 09:59, 18 August 2011

Telethonin

Telethonin crystal structure by Zou et al. (2006) interacting with Z1 and Z2 titin domains(PDB entry 1ya5)

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Proteopedia Page Contributors and Editors (what is this?)

Marcia Ivonne Peña Paz, Nikos Pinotsis, Jaime Prilusky

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