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Journal of Cell Science, Vol 109, Issue 11 2747-2754, Copyright © 1996 by Company of Biologists
JOURNAL ARTICLES |
M Gautel, D Goulding, B Bullard, K Weber and DO Furst
European Molecular Biology Laboratory, Structural Biology Division, Heidelberg, Germany.
The giant sarcomeric protein titin (also described as connectin) is composed mainly of immunoglobulin (Ig)-like and fibronectin type III (fn3)-like domains arranged consecutively. At both ends of the molecule, these domains are interrupted by sequence insertions. The amino terminus of titin is localized in the Z-disk, a structure of great variability in different muscle types. We have determined the ultrastructural position of sequences in this region of the molecule in skeletal and cardiac muscle by immunoelectron microscopy using antibodies directed against unique epitopes. Titin molecules entering the Z-disk from two half sarcomeres do not significantly overlap, showing that the amino terminus is at the centre of the Z-disk. A serine/proline rich site, which can be phosphorylated by kinases in developing muscle tissues, was identified near the amino terminus of titin. Sequence analysis revealed the presence of a novel 45 residue repeat ('Z-repeats') in this region of the molecule. The number of titin Z-repeats varies due to differential splicing. We propose that this mechanism is a means of assembling Z-disks of variable thickness and mechanical strength.
This article has been cited by other articles:
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  J. Qi, L. Chi, S. Labeit, and A. J. Banes Nuclear localization of the titin Z1Z2Zr domain and role in regulating cell proliferation Am J Physiol Cell Physiol, October 1, 2008; 295(4): C975 - C985. [Abstract] [Full Text] [PDF]
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 E. H. Lee, J. Hsin, O. Mayans, and K. Schulten Secondary and Tertiary Structure Elasticity of Titin Z1Z2 and a Titin Chain Model Biophys. J., September 1, 2007; 93(5): 1719 - 1735. [Abstract] [Full Text] [PDF]
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 H. Granzier, M. Radke, J. Royal, Y. Wu, T. C. Irving, M. Gotthardt, and S. Labeit Functional genomics of chicken, mouse, and human titin supports splice diversity as an important mechanism for regulating biomechanics of striated muscle Am J Physiol Regulatory Integrative Comp Physiol, August 1, 2007; 293(2): R557 - R567. [Abstract] [Full Text] [PDF]
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 Y. Gontier, A. Taivainen, L. Fontao, A. Sonnenberg, A. van der Flier, O. Carpen, G. Faulkner, and L. Borradori The Z-disc proteins myotilin and FATZ-1 interact with each other and are connected to the sarcolemma via muscle-specific filamins J. Cell Sci., August 15, 2005; 118(16): 3739 - 3749. [Abstract] [Full Text] [PDF]
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 B. Udd, A. Vihola, J. Sarparanta, I. Richard, and P. Hackman Titinopathies and extension of the M-line mutation phenotype beyond distal myopathy and LGMD2J Neurology, February 22, 2005; 64(4): 636 - 642. [Abstract] [Full Text] [PDF]
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M. C. Leake, D. Wilson, M. Gautel, and R. M. Simmons The Elasticity of Single Titin Molecules Using a Two-Bead Optical Tweezers Assay Biophys. J., August 1, 2004; 87(2): 1112 - 1135. [Abstract] [Full Text] [PDF]
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 W. G. Pyle and R. J. Solaro At the Crossroads of Myocardial Signaling: The Role of Z-Discs in Intracellular Signaling and Cardiac Function Circ. Res., February 20, 2004; 94(3): 296 - 305. [Abstract] [Full Text] [PDF]
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 P. Zou, M. Gautel, A. Geerlof, M. Wilmanns, M. H. J. Koch, and D. I. Svergun Solution Scattering Suggests Cross-linking Function of Telethonin in the Complex with Titin J. Biol. Chem., January 17, 2003; 278(4): 2636 - 2644. [Abstract] [Full Text] [PDF]
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 P. Young, E. Ehler, and M. Gautel Obscurin, a giant sarcomeric Rho guanine nucleotide exchange factor protein involved in sarcomere assembly J. Cell Biol., July 9, 2001; 154(1): 123 - 136. [Abstract] [Full Text] [PDF]
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H. Haravuori, A. Vihola, V. Straub, M. Auranen, I. Richard, S. Marchand, T. Voit, S. Labeit, H. Somer, L. Peltonen, et al. Secondary calpain3 deficiency in 2q-linked muscular dystrophy: Titin is the candidate gene Neurology, April 10, 2001; 56(7): 869 - 877. [Abstract] [Full Text] [PDF]
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P. van der Ven, J. Bartsch, M Gautel, H Jockusch, and D. Furst A functional knock-out of titin results in defective myofibril assembly J. Cell Sci., January 4, 2000; 113(8): 1405 - 1414. [Abstract] [PDF]
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 P. Salmikangas, O.-M. Mykkanen, M. Gronholm, L. Heiska, J. Kere, and O. Carpen Myotilin, a novel sarcomeric protein with two Ig-like domains, is encoded by a candidate gene for limb-girdle muscular dystrophy Hum. Mol. Genet., July 1, 1999; 8(7): 1329 - 1336. [Abstract] [Full Text] [PDF]
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C. C. Gregorio, K. Trombitas, T. Centner, B. Kolmerer, G. Stier, K. Kunke, K. Suzuki, F. Obermayr, B. Herrmann, H. Granzier, et al. The NH2 Terminus of Titin Spans the Z-Disc: Its Interaction with a Novel 19-kD Ligand (T-cap) Is Required for Sarcomeric Integrity J. Cell Biol., November 16, 1998; 143(4): 1013 - 1027. [Abstract] [Full Text] [PDF]
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G. Faulkner, A. Pallavicini, A. Comelli, M. Salamon, G. Bortoletto, C. Ievolella, S. Trevisan, S. Kojic', F. Dalla Vecchia, P. Laveder, et al. FATZ, a Filamin-, Actinin-, and Telethonin-binding Protein of the Z-disc of Skeletal Muscle J. Biol. Chem., December 22, 2000; 275(52): 41234 - 41242. [Abstract] [Full Text] [PDF]
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