<p>This domain is associated with telethonin, which is found at the Z-disc of sarcomeres. It is the phosphorylation target of the kinase domain of titin, and is thought to play a role in muscle development [<cite idref="PUB00034631"/>, <cite idref="PUB00035182"/>]. Deletion of the C terminus of titin, including the kinase domain, has been found to impair myofibrillogenesis [<cite idref="PUB00035183"/>]. Mutations of telethonin cause limb-girdle muscular dystrophy type 2G [<cite idref="PUB00035184"/>].</p><p>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 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 [<cite idref="PUB00040581"/>].</p><p>This entry represents the N-terminal domain.</p> Titin-like domain