TTN antibody (clone 7D3), anti-human

Background:
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. 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 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. 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. Of the many titin variants identified, five for which complete transcript information is available are described. Mutations in this gene are associated with familial hypertrophic cardiomyopathy 9 and autoantibodies to titin are produced in patients with the autoimmune disease scleroderma.

Other Names:
Titin, Connectin, Rhabdomyosarcoma antigen MU-RMS-4014, TTN

Antigen Type:
Recombinant Protein

Gene Name: TTN
Gene ID: 7273
NCBI Accession: NP_001243779.1;NP_001254479.1;NP_003310.4;NP_596869.4;NP_596870.2;NP_597676.3;NP_597681.3
Primary Accession: Q8WZ42
Other Accession: BC058824
Format: Clear, colorless solution in phosphate buffered saline, pH 7.2 .