Corpus ID: 15352243

The central Z-disk region of titin is assembled from a novel repeat in variable copy numbers.

@article{Gautel1996TheCZ,
  title={The central Z-disk region of titin is assembled from a novel repeat in variable copy numbers.},
  author={M. Gautel and D. Goulding and B. Bullard and K. Weber and D. F{\"u}rst},
  journal={Journal of cell science},
  year={1996},
  volume={109 ( Pt 11)},
  pages={
          2747-54
        }
}
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… Expand
Cardiac titin: a multifunctional giant.
TLDR
Titin's functions in the heart are discussed, with an emphasis on its role in diastolic function and the various mechanisms whereby passive stiffness can be tuned. Expand
Constitutive and variable regions of Z-disk titin/connectin in myofibril formation: a dominant-negative screen.
TLDR
Sequence analysis of chicken breast muscle titin/connectin reveals that in this tissue, where Z-disks are thin, only two Z-repeats are expressed, which supports the idea that the Z-repeat region of titin /connectin is responsible for the control Z-disk thickness as an element of highly variable length, due to extensive differential splicing. Expand
Obscurin, a giant sarcomeric Rho guanine nucleotide exchange factor protein involved in sarcomere assembly
TLDR
The presence of a calmodulin-binding IQ motif, and a Rho guanine nucleotide exchange factor domain in the COOH-terminal region suggest that obscurin is involved in Ca2+/calmodulin, as well as G protein–coupled signal transduction in the sarcomere. Expand
The NH2 Terminus of Titin Spans the Z-Disc: Its Interaction with a Novel 19-kD Ligand (T-cap) Is Required for Sarcomeric Integrity
TLDR
Using dominant-negative approaches in cardiac myocytes, both the titin Z1-Z2 domains and titin-cap are shown to be required for the structural integrity of sarcomeres, suggesting that their interaction is critical in titin filament–regulated sarcomeric assembly. Expand
Titins in C.elegans with unusual features: coiled-coil domains, novel regulation of kinase activity and two new possible elastic regions.
TLDR
There are previously unrecognized proteins in Caenorhabditis elegans that are similar to the giant muscle proteins called titins, and these are encoded by a single approximately 90kb gene, and there are multiple regions which are likely to form coiled-coil structure. Expand
Alternative splicing of an amino-terminal PEVK-like region generates multiple isoforms of Drosophila projectin.
TLDR
The completion of the projectin sequence analysis is described, which defines projectin as a 1 MDa protein, composed of 39 immunoglobulin and 39 fibronectin III domains, and leads to the identification of a domain rich in the amino acids P, E, V and K within the NH(2) terminus of projectin. Expand
Two immunoglobulin‐like domains of the Z‐disc portion of titin interact in a conformation‐dependent way with telethonin
TLDR
It is described here that the N‐terminal titin immunoglobulin domains Z1 and Z2 interact specifically with telethonin in yeast two‐hybrid analysis and protein binding assays, reflecting a possible phosphorylation regulation during myofibrillogenesis. Expand
A functional knock-out of titin results in defective myofibril assembly.
TLDR
These experiments provide the first direct evidence for the crucial role of titin in both thick filament formation as a molecular ruler and in the coordination of myofibrillogenesis. Expand
SH3 in muscles: solution structure of the SH3 domain from nebulin.
TLDR
The three-dimensional structure of the human nebulin SH3 domain in solution is determined by nuclear magnetic resonance (NMR) spectroscopy and reveals a remarkable similarity with a distinct subset of SH3 domains, especially in the structural features of the exposed hydrophobic patch that is thought to be the site of interaction with polyproline ligands. Expand
Nuclear localization of the titin Z1Z2Zr domain and role in regulating cell proliferation.
TLDR
The data implicate a dual role for titin's amino terminal region, i.e., a novel nuclear function promoting cell division in addition to its known structural role in Z-line assembly. Expand
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References

SHOWING 1-10 OF 54 REFERENCES
Towards a molecular understanding of titin.
TLDR
It is concluded that A‐band titin is likely to be involved in the ordered assembly of the vertebrate thick filament. Expand
Tertiary structure of an immunoglobulin-like domain from the giant muscle protein titin: a new member of the I set.
TLDR
The three-dimensional structure of an immunoglobulin module, located in the M-line of the sarcomere close to the titin C terminus and called 'M5', was determined by multi-dimensional NMR spectroscopy and indicates that it may be possible to use the structure of M5 as a molecular template to model most of the other immunoglOBulin-like domains in muscle titin. Expand
Phosphorylation of KSP motifs in the C‐terminal region of titin in differentiating myoblasts.
TLDR
It is suggested that titin C‐terminal phosphorylation by SP‐specific kinases is regulated during differentiation, and that this may control the assembly of M‐line proteins into regular structures during myogenesis. Expand
Titin domain patterns correlate with the axial disposition of myosin at the end of the thick filament.
TLDR
It seems likely that an alteration in the interaction between myosin and the titin fn3 domains towards the end of the thick filament is important for the formation of the crossbridge gap and thus the termination of the thin filament. Expand
Immunoglobulin-type domains of titin: same fold, different stability?
TLDR
The stability of the modules over a range of conditions was investigated by measuring key thermodynamic parameters for both thermal and chemical denaturation and by monitoring amide proton exchange as a function of time. Expand
Titins: Giant Proteins in Charge of Muscle Ultrastructure and Elasticity
TLDR
The architecture of sequences in the A band region of titin suggests why thick filament structure is conserved among vertebrates and compares two elements that correlate with tissue stiffness that suggest that titin may act as two springs in series. Expand
A molecular map of the interactions between titin and myosin-binding protein C. Implications for sarcomeric assembly in familial hypertrophic cardiomyopathy.
TLDR
The interaction of recombinant titin with overlapping fragments of human cardiac MyBP-C maps the titin-binding site within the C-terminal region, which is deleted in patients suffering from the chromosome-11-associated form of familial hypertrophic cardiomyopathy, likely to be the result of thick-filament misassembly by abolishing the ternary interaction of titin, myosin and My BP-C. Expand
The complete primary structure of human nebulin and its correlation to muscle structure.
TLDR
It is proposed that different types of nebulin molecular rulers are expressed in the different kinds of skeletal muscles by differential splicing, which is likely to explain the developmental and tissue-specific size variations of nebulins previously found in vertebrate skeletal muscles. Expand
Immunoglobulin‐type domains of titin are stabilized by amino‐terminal extension
TLDR
Results show that, while amino‐terminal extension has a profound effect on the stability of individual modules, it does not affect at all their folding pattern or their relative stabilities, and suggest that the selection of module boundaries can be of critical importance for the structural analysis of modular proteins in general. Expand
The elastic I-band region of titin is assembled in a "modular" fashion by weakly interacting Ig-like domains.
TLDR
It is suggested that folding of titin in vitro is a hierarchical event and that weak interactions between its adjacent modules must only partly account for its presumed elastic function. Expand
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