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Sarcomere assembly is regulated by the muscle protein titin. Zusätzlich bieten wir Ihnen TCAP Proteine (7) und viele weitere Produktgruppen zu diesem Protein an.
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Human Monoclonal TCAP Primary Antibody für WB - ABIN968822
Mason, Bayol, Loughna: The novel sarcomeric protein telethonin exhibits developmental and functional regulation. in Biochemical and biophysical research communications 1999
Show all 4 Pubmed References
Data show show that tcap is functionally conserved and reduction of Tcap resulted in muscular dystrophy-like phenotypes including deformed muscle structure and impaired swimming ability.
Distinct roles for telethonin N-versus C-terminus in sarcomere assembly and maintenance.
Data indicate LGMD2G protein TCAP association with limb girdle muscular dystrophy 2G (LGMD2G).
Report TCAP mutations in dilated cardiomyopathy.
cardiac telethonin is constitutively bis (zeige BAG3 Antikörper)-phosphorylated and suggest that such phosphorylation is critical for normal telethonin function, which may include maintenance of transverse tubule organization and intracellular Ca(2 (zeige CA2 Antikörper)+) transients.
This study identified no pathogenic mutations in BAG3 (zeige BAG3 Antikörper), MATR3 (zeige MATR3 Antikörper), PTRF (zeige PTRF Antikörper) or TCAP in Australian muscular dystrophy.
reduced expression of dystrophin (zeige DMD Antikörper) and titin (zeige TTN Antikörper) is associated with the pathophysiology of dilated cardiomyopathy, and TNF-alpha (zeige TNF Antikörper) may modulate the expression of these proteins via NF-kappaB (zeige NFKB1 Antikörper) pathway.
Genetic studies of tcap in zebrafish suggested that pathogenesis in LGMD2G is due to a disruption of sarcomere-T-tubular interaction, but not of sarcomere assembly per se.
review of telethonin and other new proteins of the Z-disc of skeletal muscle
telethonin protein levels seems to be at least in part regulated by neuronal activity and is thus linked to the dynamic control of myofibrillogenesis and muscle turnover in human skeletal muscle.
telethonin may play a role in linking titin (zeige TTN Antikörper) filaments at the Z-disk periphery
TCAP mutations identified which are associated with hypertrophic cardiomyopathy.
Tcap is a critical, load-sensitive regulator of t-tubule structure and function.
Telethonin knockout mice develop heart failure following biomechanical stress, owing at least in part to apoptosis of cardiomyocytes, an effect that may also play a role in human heart failure.
Whereas both promotor E boxes in Tcap are required for full activation by MyoD (zeige MYOD1 Antikörper) or myogenin (zeige MYOG Antikörper) in NIH3T3 cells, the promoter proximal E box has a greater contribution to activation in C2C12 cells and to activation by MyoD (zeige MYOD1 Antikörper) in NIH3T3 cells.
Findings suggest that TCAP knockdown with RNA interference alters normal muscle cell differentiation.
TCAP promoter can be activated by MyoD (zeige MYOD1 Antikörper), MyoG (zeige MYOG Antikörper) and MEF2 (zeige MYEF2 Antikörper) in myotubes, which indicated that TCAP may play a role in the regulation of skeletal muscle development.
Sarcomere assembly is regulated by the muscle protein titin. Titin is a giant elastic protein with kinase activity that extends half the length of a sarcomere. It serves as a scaffold to which myofibrils and other muscle related proteins are attached. This gene encodes a protein found in striated and cardiac muscle that binds to the titin Z1-Z2 domains and is a substrate of titin kinase, interactions thought to be critical to sarcomere assembly. Mutations in this gene are associated with limb-girdle muscular dystrophy type 2G.
, 19 kDa sarcomeric protein
, limb girdle muscular dystrophy 2G (autosomal recessive)
, titin cap protein
, titin-cap protein