{"product_id":"cst-19443sf","title":"CST,  19443SF, RyR1 (D4E1) Rabbit Monoclonal Antibody (BSA and Azide Free)","description":"Monoclonal Antibody for studying RyR1. Validated for Western Blotting,Immunofluorescence (Frozen). Highly specific and rigorously validated in-house, RyR1 (D4E1) Rabbit Monoclonal Antibody (BSA and Azide Free) (CST #19443) is ready to ship.\n\n\u003cb\u003eProduct Usage Information\u003c\/b\u003e\nThis product is the carrier free version of product #8153. All data were generated using the same antibody clone in the standard formulation which contains BSA and glycerol. This formulation is ideal for use with technologies requiring specialized or custom antibody labeling, including fluorophores, metals, lanthanides, and oligonucleotides. It is not recommended for ChIP, ChIP-seq, CUT\u0026amp;RUN or CUT\u0026amp;Tag assays. If you require a carrier free formulation for chromatin profiling, please contact us . Optimal dilutions\/concentrations should be determined by the end user. BSA and Azide Free antibodies are quality control tested by size exclusion chromatography (SEC) to determine antibody integrity.\n\u003cb\u003eFormulation\u003c\/b\u003e\nSupplied in 1X PBS (10 mM Na 2 HPO 4 , 3 mM KCl, 2 mM KH 2 PO 4 , and 140 mM NaCl (pH 7.8)). BSA and Azide Free. For standard formulation of this product see product # 8153\n\u003cb\u003eStorage\u003c\/b\u003e\nStore at -20°C. This product will freeze at -20°C so it is recommended to aliquot into single-use vials to avoid multiple freeze\/thaw cycles. A slight precipitate may be present and can be dissolved by gently vortexing. This will not interfere with antibody performance.\n\u003cb\u003eSpecificity \/ Sensitivity\u003c\/b\u003e\nRyR1 (D4E1) Rabbit Monoclonal Antibody (BSA and Azide Free) recognizes endogenous levels of total RyR1 protein. This antibody does not cross-react with other ryanodine receptor proteins.\nSpecies Reactivity: Human, Mouse, Rat\n\u003cb\u003eSource \/ Purification\u003c\/b\u003e\nMonoclonal antibody is produced by immunizing animals with a synthetic peptide corresponding to residues surrounding Arg830 of human RyR1 protein.\n\u003cb\u003eBackground\u003c\/b\u003e\nRyanodine receptors (RyRs) are large (\u0026gt;500 kDa), intracellular calcium channels found in the sarcoplasmic\/endoplasmic reticulum membrane and are responsible for the release of Ca from intracellular stores in excitable cells, such as muscle and neurons. RyRs exist as three mammalian isoforms (RyR1-3), all of which form homotetramers regulated by phosphorylation and\/or direct or indirect interaction with a variety of proteins (L-type calcium channels, PKA, FKBP12\/12.6, CaMKII, calmodulin, calsequestrin, junctin, and triadin) and ions (Mg and Ca ). Regulation of the RyR channel by protein modulators occurs within the large cytoplasmic domain, whereas the carboxy-terminal portion of the protein forms the ion-binding and conducting pore (1,2). RyR1 and RyR2 are predominantly expressed in skeletal and cardiac muscle, respectively, where they localize exclusively to the sarcoplasmic reticulum (SR) and facilitate calcium-mediated communication between transverse-tubules and sarcoplasmic reticulum. Contraction of skeletal muscle is triggered by release of calcium ions from the SR following depolarization of T-tubules. Research studies have shown that defects in RyR1 are the cause of malignant hyperthermia susceptibility type 1 (MHS1), central core disease of muscle (CCD), multiminicore disease with external ophthalmoplegia, and congenital myopathy with fiber-type disproportion (CFTD), each of which is manifested by defects in muscle function, metabolism, and development (2). Investigators have shown that defects in RyR2 are the cause of familial arrhythmogenic right ventricular dysplasia type 2 (ARVD2) and catecholaminergic polymorphic ventricular tachycardia type 1 (CPVT1), both of which are implicated in sudden death syndromes as a result of electrical instability and degeneration of the ventricular myocardium or stress-induced ventricular tachycardia (2). Despite low levels of expression in skeletal and smooth muscle, RyR3 is the dominant isoform in neuronal cells (hippocampal neurons, thalamus, Purkinje cells) and has been implicated in synaptic plasticity, dendritic spine remodeling, and spatial memory formation (3). The role of RyR3 in neuronal function has been substantiated by mice lacking RyR3, which demonstrate normal motor function, but possess numerous behavioral and social defects (4).\n\u003cb\u003eAlternate Names\u003c\/b\u003e\nCCO; central core disease of muscle; MHS; MHS1; PPP1R137; protein phosphatase 1, regulatory subunit 137; Ryanodine receptor 1; ryanodine receptor 1 (skeletal); ryanodine receptor type1; RYDR; RYR; RYR-1; RYR1; sarcoplasmic reticulum calcium release channel; Skeletal muscle calcium release channel; skeletal muscle ryanodine receptor; Skeletal muscle-type ryanodine receptor; SKRR; Type 1 ryanodine receptor; type 1-like ryanodine receptor\n\n\u003cb\u003eSpecification\u003c\/b\u003e\n\nREACTIVITY: H M R\nSENSITIVITY: Endogenous\nMW (kDa): ~560\nSource\/Isotype: Rabbit IgG","brand":"CST","offers":[{"title":"Default Title","offer_id":46797641613481,"sku":"19443SF","price":0.99,"currency_code":"USD","in_stock":true}],"url":"https:\/\/iright.com\/products\/cst-19443sf","provider":"Iright","version":"1.0","type":"link"}