{"product_id":"abcam-ab133646","title":"Abcam, ab133646, Anti-Telethonin antibody [EPR8375]","description":"\u003cp\u003eSize: 100µL \/ 1mL\u003cbr\u003e\nRabbit Recombinant Monoclonal Telethonin antibody. Suitable for WB and reacts with Mouse, Rat, Human samples. Cited in 5 publications.\u003cbr\u003e\nKey facts\u003cbr\u003e\nHost species:Rabbit,\u003cbr\u003e\nClonality:Monoclonal,\u003cbr\u003e\nClone number:EPR8375,\u003cbr\u003e\nIsotype:IgG,\u003cbr\u003e\nCarrier free:No,\u003cbr\u003e\nReacts with:Mouse, Rat, Human,\u003cbr\u003e\nApplications:WBSee reactivity dataSee the reactivity data table below for information on validated species and application combinations.,\u003cbr\u003e\nImmunogen:The exact immunogen used to generate this antibody is proprietary information.\u003c\/p\u003e\n\n\u003cp\u003eProduct details:\u003cbr\u003e\nPatented technology\u003cbr\u003e\nOur RabMAb\u003cbr\u003e\ntechnology is a patented hybridoma-based technology for making rabbit monoclonal antibodies. For details on our patents, please refer to\u003cbr\u003e\nRabMAb® patents\u003cbr\u003e\nWhat are the advantages of a recombinant monoclonal antibody?\u003cbr\u003e\nThis product is a recombinant monoclonal antibody, which offers several advantages including:\u003cbr\u003e\n- High batch-to-batch consistency and reproducibility\u003cbr\u003e\n- Improved sensitivity and specificity\u003cbr\u003e\n- Long-term security of supply\u003cbr\u003e\n- Animal-free batch production\u003cbr\u003e\nFor more information, read more on\u003cbr\u003e\nrecombinant antibodies\u003c\/p\u003e\n\n\u003cp\u003eProperties and Storage Information:\u003cbr\u003e\nForm-Liquid, Purity-Tissue culture supernatant, Storage buffer-pH: 7.2 - 7.4Preservative: 0.01% Sodium azideConstituents: PBS, 50% Tissue culture supernatant, 40% Glycerol (glycerin, glycerine), 0.05% BSA, Shipped at conditions-Blue Ice, Appropriate short-term storage conditions-+4°C, Appropriate long-term storage conditions--20°C, Storage information-Stable for 12 months at -20°C\u003c\/p\u003e\n\n\u003cp\u003eSupplementary Information:\u003cbr\u003e\nThis supplementary information is collated from multiple sources and compiled automatically.\u003cbr\u003e\nTelethonin also known as Tcap is a small protein with a molecular mass of approximately 19 kDa. It is an important component of the sarcomere in striated muscle cells primarily expressed in the heart and skeletal muscles. Telethonin resides at the Z-disc of the sarcomere where it interacts with titin one of the largest known proteins to maintain the structural integrity and elasticity of muscle tissues. This protein supports the mechanical stability of muscle fibers during contraction and relaxation by acting as a molecular spring.\u003cbr\u003e\nBiological function summary\u003cbr\u003e\nThe function of telethonin expands beyond simple structural support. It acts as part of the protein complex at the Z-disc important for maintaining sarcomere architecture. This complex involves interactions not only with titin but also with other structural proteins such as actin and myotilin. Telethonin's presence helps coordinate the assembly and maintenance of the Z-disc contributing to muscle strength and resilience. It also plays a role in signaling pathways that regulate muscle protein gene expression and response to mechanical stress.\u003cbr\u003e\nPathways\u003cbr\u003e\nTelethonin integrates into several significant signaling cascades. One major pathway involves the titin-telethonin interaction as part of the mechanical signaling responsible for muscle function and adaptation. Through its involvement with titin telethonin influences pathways linked to muscle hypertrophy and atrophy as well as sarcomere organization. Furthermore telethonin associates with signaling proteins like myostatin an important regulator of muscle growth modulating muscle size and adaptation to workload changes.\u003cbr\u003e\nTelethonin's malfunction often shows links with cardiomyopathies and muscular dystrophies. Mutations in the telethonin gene are associated with limb-girdle muscular dystrophy type 2G a condition where muscle weakness and degeneration occur due to defects in the muscle fiber structure. Moreover telethonin's interaction with titin connects it to dilated cardiomyopathy a condition where the heart weakens and enlarges impairing its ability to pump effectively. Understanding telethonin’s role provides insights into these muscle-related diseases and offers potential therapeutic targets for intervention.\u003c\/p\u003e","brand":"Abcam","offers":[{"title":"Default Title","offer_id":46844164898985,"sku":"ab133646","price":0.99,"currency_code":"USD","in_stock":true}],"url":"https:\/\/iright.com\/products\/abcam-ab133646","provider":"Iright","version":"1.0","type":"link"}