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Titin (connectin)

A huge actin binding protein abundant in skeletal muscle, and isoforms (smitin) exists in smooth muscles (Kim & Keller III, 2002). Titins are also minor components of some non-muscle cells (Eilersten & Keller III, 1992) and surprisingly it has been found to be a structural component of the nucleus (Machado et al, 1998) .  Titin connects a-actinin at the Z-disc in an anti-parallel fashion that seems to be important in setting up the sacromere during muscle development.


Astier, C., Raynaud, F., Lebart, M.-C., Roustan, C. and Benyamin, Y. (1998). "Binding of a native titin fragment to actin is regulated by PIP2." FEBS Letters 429, 95-98.

Atkinson, R. A., C. Joseph, et al. (2000). "Binding of a-actinin to titin: Implications for Z-disk assembly." Biochemistry 39, 5255-5264.

Eilersten, K. J. and T. C. S. Keller III (1992). “Identification and characterization of the huge protein components of the brush border cytoskeleton: Evidence for a cellular isoform of titin.” J.Cell Biol. 119(3), 549-557.

Gregorio, C. C., Granzier, H., Sorimachi, H. and Labeit, S.. (1999). "Muscle assembly: a titanic achievement?" Curr.Op.Cell Biol. 11, 18-25.

Kim, K. & Keller III, T. C. S. (2002) Smitin, a novel smooth muscle titin-like protein, interacts with myosin filaments in vivo and in vitro., JCB. 156, 101-111.

Machado, C., C. E. Sunkel, et al. (1998). "Human autoantibodies reveal titin as a chromosomal protein." J.Cell Biol. 141, 321-333.

Nwe, T. M., K. Maruyama, et al. (1999). "Relation of nebulin and connectin (titin) to dynamics of actin in nascent myofibrils of cultured skeletal muscle cells." Exp.Cell Res. 252, 33-40.

Person, V., Kostin, S., Suzuki, K., Labeit, S. & Schaper, J. (2000). "Antisense oligonucleotide experiments elucidate the essential role of titin in sarcomerogenesis in adult rat cardiomyocetes in long-term culture." J.Cell Sci. 113, 3851-3859.

Satoh, M., M. Takahashi, et al. (1999). "Structural analysis of the titin gene in hypertrophic cardiomyopathy: Identifiaction of a novel disease gene." BBRC 262, 411-417.

Sorimachi, H., A. Freiburg, et al. (1997). "Tissue-specific expression and a-actinin binding properties of the Z-disc titin: Implications for the nature of vertebrate Z-discs." J.Mol.Biol. 270, 688-695.

Trinick, J. and L. Tskhovrebova (1999). "Titin: a molecular control freak." Trends Cell Biol. 9, 377-380.

Tskhovrebova, L., J. Trinick, et al. (1997). "Elasticity and unfolding of single molecules of the giant muscle protein titin." Nature 387, 308-312.

van der Ven, P. F., J. W. Bartsch, et al. (2000). "A functional knock-out of titin results in defective myofibril assembly." J.Cell Sci. 113, 1405-1414.

van Straaten, M., D. Goulding, et al. (1999). "Association of kettin with actin in the Z-disc of insect flight muscle." J.Mol.Biol. 285, 1549-1562.

Young, P., C. Ferguson, et al. (1998). "Molecular structure of the sarcomeric Z-disk: two types of titin interactions lead to an asymetric sorting of a-actinin." EMBO J. 17(6), 1614-1624.

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