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titin

MIM.188840

Friday 14 November 2003

The central sarcomeric protein titin is encoded by the gene TTN at 2q31.

Functions

 complexes associated with the myofibrillar protein titin sense myocyte stretch.

Pathology (titinopathy)

 mutations in the tibial muscular dystrophy (MIM.600334) (12145747)
 mutations in dilated cardiomyopathy

References

 Miller MK, Granzier H, Ehler E, Gregorio CC. The sensitive giant: the role of titin-based stretch sensing complexes in the heart. Trends Cell Biol. 2004 Mar;14(3):119-26. PMID: 15003620

 Hein S, Schaper J. Weakness of a giant: mutations of the sarcomeric protein titin. Trends Mol Med. 2002 Jul;8(7):311-3. PMID: 12114104