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GoPubMed Proteins lists recent and important papers and reviews for proteins. Page last changed on 29 Mar 2014.

Titin

titin
This gene encodes a large abundant protein of striated muscle. The product of this gene is divided into two regions, a N-terminal I-band and a C-terminal A-band. The I-band, which is the elastic part of the molecule, contains two regions of tandem immunoglobulin domains on either side of a PEVK region that is rich in proline, glutamate, valine and lysine. The A-band, which is thought to act as a protein-ruler, contains a mixture of immunoglobulin and fibronectin repeats, and possesses kinase activity. An N-terminal Z-disc region and a C-terminal M-line region bind to the Z-line and M-line of the sarcomere, respectively, so that a single titin molecule spans half the length of a sarcomere. Titin also contains binding sites for muscle associated proteins so it serves as an adhesion template for the assembly of contractile machinery in muscle cells. It has also been identified as a structural protein for chromosomes. Alternative splicing of this gene results in multiple transcript variants. Considerable variability exists in the I-band, the M-line and the Z-disc regions of titin. Variability in the I-band region contributes to the differences in elasticity of different titin isoforms and, therefore, to the differences in elasticity of different muscle types. Mutations in this gene are associated with familial hypertrophic cardiomyopathy 9, and autoantibodies to titin are produced in patients with the autoimmune disease scleroderma. [provided by RefSeq, Feb 2012] (from NCBI)
Top mentioned proteins: CAN, Actin, HAD, nebulin, V1a
Papers using titin antibodies
Obscurin, a giant sarcomeric Rho guanine nucleotide exchange factor protein involved in sarcomere assembly
Supplier
Gautel Mathias et al., In The Journal of Cell Biology, 1998
... For titin total human cardiac cDNA (CLONTECH Laboratories, Inc.) was used ...
M line–deficient titin causes cardiac lethality through impaired maturation of the sarcomere
Supplier
Gotthardt Michael et al., In The Journal of Cell Biology, 1997
... Generation of titin M-line knockout miceThe transgenic mice with loxP sites ...
Papers on titin
Gigantic business: titin properties and function through thick and thin.
New
Hamdani et al., Bochum, Germany. In Circ Res, 14 Apr 2014
TTN, which encodes titin, is also a major human disease gene.
S-glutathionylation of cryptic cysteines enhances titin elasticity by blocking protein folding.
New
Impact
Fernández et al., New York City, United States. In Cell, 13 Apr 2014
The giant elastic protein titin is a determinant factor in how much blood fills the left ventricle during diastole and thus in the etiology of heart disease.
CIS-Splicing and Translation of the Pre-Trans-Splicing Molecule Combine with Efficiency in Spliceosome-Mediated RNA Trans-Splicing.
New
Charton et al., Rouen, France. In Mol Ther, 12 Apr 2014
This issue concerns, in particular, dysferlinopathies and titinopathies that are due to mutations in the dysferlin (DYSF) and titin (TTN) genes.
Hereditary myopathy with early respiratory failure is associated with misfolding of the titin fibronectin III 119 subdomain.
New
Macao et al., Göteborg, Sweden. In Neuromuscul Disord, 15 Mar 2014
The disease is associated with mutations in the titin gene (TTN).
The physiological role of cardiac cytoskeleton and its alterations in heart failure.
Review
New
van der Velden et al., Amsterdam, Netherlands. In Biochim Biophys Acta, 28 Feb 2014
The role of contractile thick and thin filament proteins, the elastic protein titin, and their anchorage at the Z-disc and M-band, with associated proteins are reviewed in physiologic and pathologic conditions leading to heart failure.
Gene expression profiling in tibial muscular dystrophy reveals unfolded protein response and altered autophagy.
New
Udd et al., Helsinki, Finland. In Plos One, Dec 2013
Tibial muscular dystrophy (TMD) is a late onset, autosomal dominant distal myopathy that results from mutations in the two last domains of titin.
Transcriptional analysis of the porcine TTID gene and association of different TTID genotypes with carcass traits.
New
Deng et al., Wuhan, China. In Genet Mol Res, Dec 2013
The titin immunoglobulin domain (TTID) protein localizes to the Z line in muscle and binds to alpha-actinin and gamma-filamin.
High-speed force spectroscopy unfolds titin at the velocity of molecular dynamics simulations.
New
Impact
Scheuring et al., Marseille, France. In Science, Dec 2013
The mechanical unfolding of the muscle protein titin by atomic force microscopy was a landmark in our understanding of single-biomolecule mechanics.
What's in the literature?
Review
New
El-Dokla, Pittsburgh, United States. In J Clin Neuromuscul Dis, Sep 2013
According to 2 recent reports, it seems that titin mutations may be an underrecognized cause of myopathy with early respiratory failure in adults.
Mechanistic and functional diversity in the mechanosensory kinases of the titin-like family.
Review
New
Rigden et al., Liverpool, United Kingdom. In Biochem Soc Trans, Aug 2013
The giant cytoskeletal kinases of the titin-like family are emerging as key mediators of stretch-sensing in muscle.
Mutational heterogeneity in cancer and the search for new cancer-associated genes.
New
Impact
Getz et al., Cambridge, United States. In Nature, Aug 2013
The list includes many implausible genes (such as those encoding olfactory receptors and the muscle protein titin), suggesting extensive false-positive findings that overshadow true driver events.
Structure of giant muscle proteins.
Review
Wright et al., Harrisonburg, United States. In Front Physiol, 2012
UNLABELLED: Giant muscle proteins (e.g., titin, nebulin, and obscurin) play a seminal role in muscle elasticity, stretch response, and sarcomeric organization.
Intracellular Regulation of Matrix Metalloproteinase-2 Activity: New Strategies in Treatment and Protection of Heart Subjected to Oxidative Stress.
Review
Sawicki, Saskatoon, Canada. In Scientifica (cairo), 2012
One such mechanism involves the proteolytic degradation of contractile proteins, such as troponin I (TnI), myosin heavy chain, titin, and the myosin light chains (MLC1 and MLC2) by matrix metalloproteinase-2 (MMP-2).
A novel mechanism involving four-and-a-half LIM domain protein-1 and extracellular signal-regulated kinase-2 regulates titin phosphorylation and mechanics.
GeneRIF
Sheikh et al., San Diego, United States. In J Biol Chem, 2012
A novel mechanism involving four-and-a-half LIM domain protein-1 and extracellular signal-regulated kinase-2 regulates titin phosphorylation and mechanics.
Independent evolution of striated muscles in cnidarians and bilaterians.
Impact
Technau et al., Vienna, Austria. In Nature, 2012
Cnidarians and ctenophores possess striated muscle myhc orthologues but lack crucial components of bilaterian striated muscles, such as genes that code for titin and the troponin complex, suggesting the convergent evolution of striated muscles.
Spontaneous dimerization of titin protein Z1Z2 domains induces strong nanomechanical anchoring.
GeneRIF
Fernández et al., London, United Kingdom. In J Biol Chem, 2012
Spontaneous dimerization of titin protein Z1Z2 domains induces strong nanomechanical anchoring.
Titin mutation segregates with hereditary myopathy with early respiratory failure.
GeneRIF
Chinnery et al., Newcastle upon Tyne, United Kingdom. In Brain, 2012
This study presented that patients with hereditary myopathy with early respiratory failure linke with Titin mutation.
Hereditary myopathy with early respiratory failure associated with a mutation in A-band titin.
GeneRIF
Oldfors et al., Göteborg, Sweden. In Brain, 2012
This study identified three different Swedish Hereditary myopathy with early respiratory failure families with a new mutation in the A-band titin.
The Sjögren-Larsson syndrome gene encodes a hexadecenal dehydrogenase of the sphingosine 1-phosphate degradation pathway.
GeneRIF
Kihara et al., Sapporo, Japan. In Mol Cell, 2012
the Sjogren-Larsson syndrome-causative gene ALDH3A2 is responsible for conversion of the sphingosine 1-phosphate degradation product hexadecenal to hexadecenoic acid
RBM20, a gene for hereditary cardiomyopathy, regulates titin splicing.
Impact
GeneRIF
Gotthardt et al., Madison, United States. In Nat Med, 2012
In addition to titin (TTN), we identified a set of 30 genes with conserved splicing regulation between humans and rats
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