For Research Use Only. Not for use in diagnostic procedures.

Monoclonal

Mouse

500ug/ml (lot specific)

Manufactured in an ISO 9001:2008 and ISO 13485:2003 Certified Laboratory.

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Small volumes of anti-TTN antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

Western Blot (WB)

3,831,069 Da

titin

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 s 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]

Titin: a protein kinase that is abundant in striated muscle. 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. A 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 s 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. Mutations are associated with familial hypertrophic cardiomyopathy 9 and autoantibodies to titin are produced in patients with the autoimmune disease scleroderma.

Protein type: CAMK group; EC 2.7.11.1; Kinase, protein; MLCK family; Protein kinase, CAMK; Protein kinase, Ser/Thr (non-receptor)

Chromosomal Location of Human Ortholog: 2q31.2

Cellular Component: condensed nuclear chromosome; cytosol; extracellular exosome; extracellular region; I band; M band; muscle myosin complex; Z disc

Molecular Function: actin filament binding; actinin binding; ATP binding; calcium ion binding; calmodulin binding; enzyme binding; identical protein binding; muscle alpha-actinin binding; protease binding; protein binding; protein kinase binding; protein self-association; protein serine/threonine kinase activity; protein tyrosine kinase activity; structural constituent of muscle; telethonin binding

Biological Process: cardiac muscle contraction; cardiac muscle fiber development; cardiac muscle hypertrophy; cardiac muscle morphogensis; cardiac myofibril assembly; detection of muscle stretch; mitotic chromosome condensation; muscle contraction; muscle filament sliding; muscle thin filament assembly; peptidyl-tyrosine phosphorylation; platelet degranulation; positive regulation of gene expression; positive regulation of protein secretion; protein kinase A signaling; regulation of catalytic activity; regulation of protein kinase activity; response to calcium ion; sarcomere organization; sarcomerogenesis; skeletal muscle myosin thick filament assembly; striated muscle contraction

Disease: Cardiomyopathy, Dilated, 1g; Hereditary Myopathy With Early Respiratory Failure; Muscular Dystrophy, Limb-girdle, Type 2j; Myopathy, Early-onset, With Fatal Cardiomyopathy; Tibial Muscular Dystrophy, Tardive

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