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GoPubMed Proteins lists recent and important papers and reviews for proteins. Page last changed on 21 Jan 2015.


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
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
Elastic Proteins in the Flight Muscle of Manduca sexta.
Irving et al., Chicago, United States. In Arch Biochem Biophys, 17 Feb 2015
Analysis of the gene sequence showed that all 4 putative Sallimus and kettin isoforms could be explained as products of alternative splicing of the single sls gene.
Excessive degradation of adenine nucleotides by up-regulated AMP deaminase underlies afterload-induced diastolic dysfunction in the type 2 diabetic heart.
Miura et al., Sapporo, Japan. In J Mol Cell Cardiol, 16 Feb 2015
In contrast, PVRs under cardioplegic arrest in buffer-perfused isolated hearts were similar in OLETF and LETO, indicating that extracellular matrix or titin remodeling does not contribute to the afterload-induced increase in stiffness of the beating ventricle of OLETF.
Tackling the achilles' heel of genetic testing.
Watkins, Oxford, United Kingdom. In Sci Transl Med, 14 Feb 2015
Assigning pathogenicity to rare genetic variants is at its hardest with the enormous titin gene, but comprehensive genomic analysis makes the task more tractable (Roberts et al., this issue).
Integrated allelic, transcriptional, and phenomic dissection of the cardiac effects of titin truncations in health and disease.
Cook et al., Boston, United States. In Sci Transl Med, 14 Feb 2015
The recent discovery of heterozygous human mutations that truncate full-length titin (TTN, an abundant structural, sensory, and signaling filament in muscle) as a common cause of end-stage dilated cardiomyopathy (DCM) promises new prospects for improving heart failure management.
Syndecan-4 is a key determinant of collagen cross-linking and passive myocardial stiffness in the pressure-overloaded heart.
Christensen et al., Oslo, Norway. In Cardiovasc Res, 12 Feb 2015
Disruption of titin anchoring by salt extraction of actin and myosin filaments revealed that the effect of syndecan-4 on passive tension was due to extracellular matrix remodeling.
Sarcomeric protein isoform transitions in cardiac muscle: A journey to heart failure.
Guo et al., Laramie, United States. In Biochim Biophys Acta, 31 Jan 2015
In this mini-review, we summarized isoform transitions of several most widely examined sarcomeric proteins including myosin, actin, troponin, tropomyosin, titin and myosin binding protein-C, and the consequence of these abnormal isoform transitions.
The Structure and Regulation of Human Muscle α-Actinin.
Djinović-Carugo et al., Vienna, Austria. In Cell, 04 Jan 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.
Genetic basis of limb-girdle muscular dystrophies: the 2014 update.
Savarese et al., Napoli, Italy. In Acta Myol, May 2014
The autosomal recessive forms (LGMD2) are: LGMD2A (calpain 3), LGMD2B (dysferlin), LGMD2C (γ sarcoglycan), LGMD2D (α sarcoglycan), LGMD2E (β sarcoglycan), LGMD2F (δ sarcoglycan), LGMD2G (telethonin), LGMD2H (TRIM32), LGMD2I (FKRP), LGMD2J (titin), LGMD2K (POMT1), LGMD2L (anoctamin 5), LGMD2M (fukutin), LGMD2N (POMT2), LGMD2O (POMTnG1), LGMD2P (dystroglycan), LGMD2Q (plectin), LGMD2R (desmin), LGMD2S (TRAPPC11), LGMD2T (GMPPB), LGMD2U (ISPD), LGMD2V (Glucosidase, alpha ), LGMD2W (PINCH2).
S-glutathionylation of cryptic cysteines enhances titin elasticity by blocking protein folding.
Fernández et al., New York City, United States. In Cell, 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.
Pathogenic mechanisms in centronuclear myopathies.
Gautel et al., London, United Kingdom. In Front Aging Neurosci, 2013
The most common forms of congenital myopathies with central nuclei have been attributed to X-linked recessive mutations in the MTM1 gene encoding myotubularin ("X-linked myotubular myopathy"), autosomal-dominant mutations in the DNM2 gene encoding dynamin-2 and the BIN1 gene encoding amphiphysin-2 (also named bridging integrator-1, BIN1, or SH3P9), and autosomal-recessive mutations in BIN1, the RYR1 gene encoding the skeletal muscle ryanodine receptor, and the TTN gene encoding titin.
High-speed force spectroscopy unfolds titin at the velocity of molecular dynamics simulations.
Scheuring et al., Marseille, France. In Science, 2013
The mechanical unfolding of the muscle protein titin by atomic force microscopy was a landmark in our understanding of single-biomolecule mechanics.
[Comparative studies of amyloid properties of muscles proteins and brain Abeta-peptides and identification of approaches to destruction of their amyloids in vitro].
Podlubnaya et al., In Biofizika, 2013
In this review our data on the comparative study of amyloid properties of titin family proteins and brain Abeta-peptides are represented.
Mutational heterogeneity in cancer and the search for new cancer-associated genes.
Getz et al., Cambridge, United States. In Nature, 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.
Three-dimensional structure of the human myosin thick filament: clinical implications.
Al-Khayat, Doha, Qatar. In Glob Cardiol Sci Pract, 2012
titin and myosin binding protein C) are known to be associated with a number of familial human cardiomyopathies (e.g.
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.
Independent evolution of striated muscles in cnidarians and bilaterians.
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.
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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