TTN
titin
Sinónimos:
CMD1G, CMH9, CMPD4, EOMFC, HMERF, LGMD2J, MYLK5, TMD
Especie:
Human TTN (7273), Mouse Ttn (22138), Rat Ttn (84015), chicken TTN (424126), dog TTN (478819), dog TTN (478820), domestic cat TTN (101080315), domestic cat TTN (751825), cow TTN (540561), Domestic Rabbit TTN (100008570) ...Más
ID UniProtKB:
ID del gen:
- Human(7273) Summary: 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]
- Mouse(22138) titin
- Rat(84015) titin
- chicken(424126) titin
- dog(478819) titin
- dog(478820) titin
- domestic cat(101080315) titin
- domestic cat(751825) titin
- cow(540561) titin
- Domestic Rabbit(100008570) titin
- sheep(101122837) titin
- domestic guinea pig(100722767) titin
- naked mole-rat(101717863) titin
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Antibodies
Referencia del producto
Descripción
Reactividad de especies
Aplicación
Anti-Titin (NT/Z-domain) Antibody, clone 17D11.1, clone 17D11.1, from mouse,
Reactividad de especies
human, mouse
Aplicación
immunohistochemistry
western blot
western blot
Anti-Titin Antibody, clone 9B9, culture supernatant, clone 9B9, Chemicon®,
Reactividad de especies
human
Aplicación
immunohistochemistry
western blot
western blot