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  Domain Name: Telethonin
Telethonin protein. Telethonin is a 167-residue protein which complexes with the large muscle protein, titin. The very N-terminus of titin, composed of two immunoglobulin-like (Ig) domains, referred to as Z1 and Z2, interacts with the N-terminal region (residues 1-53) of telethonin, mediating the antiparallel assembly of two Z1Z2 domains. The C-terminus of the telethonin appears to induce dimerisation of this 2:1 titin/telethonin structure which thus forms a complex necessary for myofibril assembly and maintenance of the intact Z-disk of skeletal and cardiac muscles.
No pairwise interactions found for the domain Telethonin

Total Mutations Found: 6
Total Disease Mutations Found: 5
This domain occurred 1 times on human genes (2 proteins).



  CARDIOMYOPATHY, DILATED 1N (CMD1N)
  CARDIOMYOPATHY, DILATED, 1N
  CARDIOMYOPATHY, FAMILIAL HYPERTROPHIC (CMH)
  MUSCULAR DYSTROPHY, LIMB-GIRDLE, TYPE 2G


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Range on the Protein:  

   Protein ID            Protein Position

Domain Position:  


No Conserved Features/Sites Found for Telethonin









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Please Cite: Peterson, T.A., Adadey, A., Santana-Cruz ,I., Sun, Y., Winder A, Kann, M.G., (2010) DMDM: Domain Mapping of Disease Mutations. Bioinformatics 26 (19), 2458-2459.

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