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GoPubMed Proteins lists recent and important papers and reviews for proteins. Page last changed on 25 Jan 2016.


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, Calpain
Papers using titin antibodies
Obscurin, a giant sarcomeric Rho guanine nucleotide exchange factor protein involved in sarcomere assembly
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
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
The effect of electrical stimulation on post mortem myofibrillar protein degradation and small heat shock protein kinetics in bull beef.
Farouk et al., Piracicaba, Brazil. In Meat Sci, 31 Mar 2016
High pHu meat tenderised faster which corresponded with the faster degradation of titin and desmin in this group compared with low and intermediate pHu meat.
Relevance of Truncating Titin Mutations in Dilated Cardiomyopathy.
Koskenvuo et al., Helsinki, Finland. In Clin Genet, 18 Feb 2016
UNASSIGNED: Dilated cardiomyopathy (DCM), a genetically heterogeneous cardiac disease characterized by left ventricular dilatation and systolic dysfunction, is caused majorly by truncations of titin (TTN), especially in A-band region.
Titin-Based Nanoparticle Tension Sensors Map High-Magnitude Integrin Forces within Focal Adhesions.
Salaita et al., Atlanta, United States. In Nano Lett, 13 Feb 2016
Herein we challenge integrins with the most mechanically stable molecular tension probe, which is comprised of the immunoglobulin 27th (I27) domain of cardiac titin flanked with a fluorophore and gold nanoparticle.
Shared Genetic Predisposition in Peripartum and Dilated Cardiomyopathies.
IMAC-2 and IPAC Investigators et al., Singapore, Singapore. In N Engl J Med, 06 Feb 2016
UNASSIGNED: Background Peripartum cardiomyopathy shares some clinical features with idiopathic dilated cardiomyopathy, a disorder caused by mutations in more than 40 genes, including TTN, which encodes the sarcomere protein titin.
Monovalent Strep-Tactin for strong and site-specific tethering in nanospectroscopy.
Pippig et al., München, Germany. In Nat Nanotechnol, 31 Jan 2016
Using titin kinase and green fluorescent protein, we show that monovalent Strep-Tactin is generally applicable to protein unfolding experiments.
Molecular and structural transition mechanisms in long-term volume overload.
Toischer et al., Göttingen, Germany. In Eur J Heart Fail, 23 Jan 2016
Maladaptive remodelling resulted in significantly reduced sarcomeric titin phosphorylation (causing increased sarcomeric stiffness), whereas interstitial fibrosis was not increased.
Comparative mass spectrometric and immunoassay-based proteome analysis in serum of Duchenne muscular dystrophy patients.
van der Burgt et al., Leiden, Netherlands. In Proteomics Clin Appl, 17 Jan 2016
Previously reported biomarkers, such as muscle-derived titin, myosin and carbonic anhydrase I (CA1), were verified.
A new titinopathy: Childhood-juvenile onset Emery-Dreifuss-like phenotype without cardiomyopathy.
Richard et al., Helsinki, Finland. In Neurology, 15 Jan 2016
The identification of a homozygous truncating mutation in the M-line part of titin prompted us to sequence this region in 2 additional patients presenting similar clinical and biochemical characteristics.
Limb-Girdle Muscular Dystrophy Type 2A Resulting From c.C479G and c.G1818A Mutations in the Calpain-3 Gene.
Vélez et al., San Juan, Puerto Rico. In J Clin Neuromuscul Dis, 31 Dec 2015
Whereas the novel mutation c.G1818A seems to affect mRNA translation of the protein region involved in titin binding.
HEART DISEASE. Titin mutations in iPS cells define sarcomere insufficiency as a cause of dilated cardiomyopathy.
Seidman et al., Boston, United States. In Science, Sep 2015
Human mutations that truncate the massive sarcomere protein titin [TTN-truncating variants (TTNtvs)] are the most common genetic cause for dilated cardiomyopathy (DCM), a major cause of heart failure and premature death.
The genetic landscape of cardiomyopathy and its role in heart failure.
Puckelwartz et al., Chicago, United States. In Cell Metab, Mar 2015
Dilated cardiomyopathy is often triggered by mutations that disrupt the giant protein titin.
The structure and regulation of human muscle α-actinin.
Djinović-Carugo et al., Vienna, Austria. In Cell, 2015
The structure provides insight into the phosphoinositide-based mechanism controlling its interaction with sarcomeric proteins such as titin, lays a foundation for studying the impact of pathogenic mutations at molecular resolution, and is likely to be broadly relevant for the regulation of spectrin-like proteins.
Transient misfolding dominates multidomain protein folding.
Schuler et al., Zürich, Switzerland. In Nat Commun, 2014
Here we combine microfluidic-mixing single-molecule kinetics, ensemble experiments and molecular simulations to investigate how misfolding between the immunoglobulin-like domains of titin is prevented.
Role of Titin Missense Variants in Dilated Cardiomyopathy.
Familial Cardiomyopathy Registry et al., Tucson, United States. In J Am Heart Assoc, 2014
BACKGROUND: The titin gene (TTN) encodes the largest human protein, which plays a central role in sarcomere organization and passive myocyte stiffness.
Prevalence of Titin Truncating Variants in General Population.
Alastalo et al., Helsinki, Finland. In Plos One, 2014
BACKGROUND: Truncating titin (TTN) mutations, especially in A-band region, represent the most common cause of dilated cardiomyopathy (DCM).
A novel mechanism involving four-and-a-half LIM domain protein-1 and extracellular signal-regulated kinase-2 regulates titin phosphorylation and mechanics.
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.
Spontaneous dimerization of titin protein Z1Z2 domains induces strong nanomechanical anchoring.
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.
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.
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.
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
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