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proteins. Page last changed on 19 Dec 2016.
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)
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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
Supplier
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
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.
New
Piracicaba, Brazil. In Meat Sci, 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.
New
Helsinki, Finland. In Clin Genet, 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.
Shared Genetic Predisposition in Peripartum and Dilated Cardiomyopathies.
New
Impact
Singapore, Singapore. In N Engl J Med, 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.
New
Impact
München, Germany. In Nat Nanotechnol, 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.
New
Göttingen, Germany. In Eur J Heart Fail, 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.
New
Leiden, Netherlands. In Proteomics Clin Appl, Jan 2016
Previously reported biomarkers, such as muscle-derived titin, myosin and carbonic anhydrase I (CA1), were verified.
Resuscitation of a dead cardiomyocyte.
Review
New
Louisville, United States. In Heart Fail Rev, Nov 2015
The activation of calpains beyond the calpastatin-mediated inhibition due to extensive calcium harbor can lead to titin degradation, damage to the sarcomere and contractile dysfunction.
The Rapidly Evolving Role of Titin in Cardiac Physiology and Cardiomyopathy.
Review
New
Calgary, Canada. In Can J Cardiol, Nov 2015
Truncating variants in TTN have been reported in about 25% of patients with DCM and in 2%-3% of controls; however, most of the disease-associated truncation variants were found in constitutively expressed exons across the gene and in A-band titin, which is abundant in both major cardiac isoforms N2B and N2BA.
Structural advances on titin: towards an atomic understanding of multi-domain functions in myofilament mechanics and scaffolding.
Review
New
Liverpool, United Kingdom. In Biochem Soc Trans, Nov 2015
Titin is a gigantic filamentous protein of the muscle sarcomere that plays roles in myofibril mechanics and homoeostasis.
Myofibrillar myopathies: State of the art, present and future challenges.
Review
New
Paris, France. In Rev Neurol (paris), Oct 2015
The boundaries of this concept are still uncertain, and whereas six genes (DES, CRYAB, LDB3/ZASP, MYOT, FLNC and BAG3) are now classically considered as responsible for MFM, other entities such as FHL1 myopathy or Hereditary Myopathy with Early Respiratory Failure linked to mutations of titin can now as well be included in this group.
An update on laboratory diagnosis in myasthenia gravis.
Review
New
Vancouver, Canada. In Clin Chim Acta, Oct 2015
They also broach over the striated antibodies, less frequent and clinically less useful such as anti-titin, -ryanodine, -agrin and -rapsyn.
HEART DISEASE. Titin mutations in iPS cells define sarcomere insufficiency as a cause of dilated cardiomyopathy.
New
Impact
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.
Review
New
Impact
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.
Impact
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.
Prevalence of Titin Truncating Variants in General Population.
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.
GeneRIF
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.
GeneRIF
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
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
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
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
