• merck millipore,默克密理博,MAB3086,Anti-Ryanodine Receptor Antibody, clone RYR.1
  • merck millipore,默克密理博,MAB3086,Anti-Ryanodine Receptor Antibody, clone RYR.1

    产品名称:Anti-Ryanodine Receptor Antibody, clone RYR.1
    产品型号:MAB3086
    Anti-Ryanodine Receptor Antibody, clone RYR.1 detects level of Ryanodine Receptor & has been published & validated for use in ELISA, FC, IC, IH, IP, RIA & WB.

    merck millipore,默克密理博,MAB3086,Anti-Ryanodine Receptor Antibody, clone RYR.1

  • 产品介绍
  • merck millipore,默克密理博,MAB3086,Anti-Ryanodine Receptor Antibody, clone RYR.1

    重要规格表

    品种反应性 主要应用 宿主 格式 抗体类型
    R, B, Ca, H, M, PoWB, ELISA, FC, ICC, IHCMPurifiedMonoclonal Antibody
    描述
    产品目录编号 MAB3086
    品牌系列 Chemicon®
    商名
    • Chemicon
    描述 Anti-Ryanodine Receptor Antibody, clone RYR.1
    产品信息
    格式 Purified
    演示 Purified immunoglobulin from culture supernatant. In 10 mM sodium phosphate, 150 mM NaCl, 0.01% NaN3, pH. 7.5
    应用
    应用 Anti-Ryanodine Receptor Antibody, clone RYR.1 detects level of Ryanodine Receptor & has been published & validated for use in ELISA, FC, IC, IH, IP, RIA & WB.
    主要应用
    • Western Blotting
    • ELISA
    • Flow Cytometry
    • Immunocytochemistry
    • Immunohistochemistry
    应用说明 Immunoblot: 1:100 using enhanced chemiluminescence detection. It is suggested that you perform a dot blot to verify presence of the RyR protein if you are having difficulties performing an immunoblot assay.

    Immunocytochemistry: 1:100 by indirect immunofluorescence. Visualization by confocal microscopy is required, as detection by standard fluorescent microscopy will not be adequate to detect the RyR. Additionally, fluorescent, not enzymatic, detection is required. Due to the intensity of confocal lasers, use of an anti-fading agent, such as DABCO, is strongly recommended.

    Immunoprecipitation: 1:100

    Flow cytometry: 1:500

    ELISA: 1:1000

    Immunohistochemistry: 1:100

    RIA: 1:5000

    Optimal working dilutions must be determined by the end user.
    生物信息
    免疫原品种 Short synthetic polypeptide corresponding to the c-terminal domain of the ryanodine receptor.
    克隆 RYR.1
    浓缩 Please refer to the Certificate of Analysis for the lot-specific concentration.
    宿主 Mouse
    特异性 Reacts with the C-terminus cytoplasmic domain of ryanodine receptor. Ryanodine receptors have been shown to play critical roles in the intracellular Ca2+ signaling occurring during cell activation in muscle cells and non-muscle cells. MAB3086 reacts with ryanodine receptor (MW approx. 500kDa) isolated from a variety of cell types (e.g. lymphocytes, macrophages, granulocytes, fibroblasts, epithelial, endothelial cells, skeletal muscle, cardiac muscle and brain tissues).
    同种型 IgG2b
    品种反应性 RatBovineCanineHumanMousePig
    抗体类型 Monoclonal Antibody
    Entrez基因编号
    • NM_001042723.1
    • NM_000540.2
    Entrez基因汇总 This gene encodes a ryanodine receptor found in skeletal muscle. The encoded protein functions as a calcium release channel in the sarcoplasmic reticulum but also serves to connect the sarcoplasmic reticulum and transverse tubule. Mutations in this gene are associated with malignant hyperthermia susceptibility, central core disease, and minicore myopathy with external ophthalmoplegia. Alternatively spliced transcripts encoding different isoforms have been described.
    基因符号
    • RYR-1
    • RYR1
    • CCO
    • RyR1
    • SKRR
    • RYR
    • MHS1
    • MHS
    • RYDR
    • CCD
    UniProt编号
    • P21817
    UniProt汇总 FUNCTION: SwissProt: P21817 # Communication between transverse-tubules and sarcoplasmic reticulum. Contraction of skeletal muscle is triggered by release of calcium ions from SR following depolarization of T-tubules.
    SIZE: 5038 amino acids; 565176 Da
    SUBUNIT: Homotetramer (Potential).
    SUBCELLULAR LOCATION: Membrane; Multi-pass membrane protein (Probable).
    TISSUE SPECIFICITY: Skeletal muscle and brain (cerebellum and hippocampus).
    DISEASE: SwissProt: P21817 # Defects in RYR1 are a cause of malignant hyperthermia (MH) [MIM:145600]. MH is an autosomal dominant pharmacogenetic disorder of skeletal muscle and is one of the main causes of death due to anesthesia. In susceptible people, an MH episode can be triggered by all commonly used inhalational anesthetics such as halothane and by depolarizing muscle relaxants such as succinylcholine. The clinical features of the myopathy are hyperthermia, accelerated muscle metabolism, contractures, metabolic acidosis, tachycardia and death, if not treated with the postsynaptic muscle relaxant, dantrolene. Susceptibility to MH can be determined with the in vitro contracture test (IVCT): observing the magnitude of contractures induced in strips of muscle tissue by caffeine alone and halothane alone. Patients with normal response are MH normal (MHN), those with abnormal response to caffeine alone or halothane alone are MH equivocal (MHE(C) and MHE(H) respectively). & Defects in RYR1 are a cause of central core disease of muscle (CCD) [MIM:117000]. CCD is an autosomal dominant congenital myopathy. Both clinical and histological variability is observed, but affected individuals typically display hypotonia and proximal muscle weakness in infancy, leading to the delay of motor milestones. The clinical course of the disorder is usually slow or nonprogressive in adulthood, and the severity of the symptoms may vary from normal to significant muscle weakness. Microscopic examination of CCD-affected skeletal muscle reveals a predominance of type I fibers containing amorphous-looking areas (cores) that do not stain with oxidative and phosphorylase histochemical techniques. & Defects in RYR1 are the cause of multiminicore disease with external ophthalmoplegia (MMDO) [MIM:255320]; also known as multicore myopathy with external ophthalmoplegia or minicore myopathy with external ophthalmoplegia. MMD is a clinically heterogeneous neuromuscular disorder. General features include neonatal hypotonia, delayed motor development, and generalized muscle weakness and amyotrophy, which may progress slowly or remain stable. Muscle biopsy shows multiple, poorly circumscribed, short areas of sarcomere disorganization and mitochondria depletion (areas termed minicores) in most muscle fibers. Typically, no dystrophic signs, such as muscle fiber necrosis or regeneration or significant endomysial fibrosis, are present in multiminicore disease.
    SIMILARITY: SwissProt: P21817 ## Belongs to the ryanodine receptor family. & Contains 3 B30.2/SPRY domains. & Contains 5 MIR domains.
    MISCELLANEOUS: The calcium release channel is modulated by calcium ions, magnesium ions, ATP and calmodulin. & The calcium release channel activity resides in the C-terminal region while the remaining part of the protein constitutes the 'foot' structure spanning the junctional gap between the SR and the T-tubule. It is possible that the foot structure interacts with the cytoplasmic region of the dihydropyridine receptor. & Ryanodine is an alkaloid that binds to the Ca- release channel in junctional SR and modulates its activity.
    产品使用声明
    使用声明
    • Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.
    储存和货运信息
    存储条件 Maintain at -20°C in undiluted aliquots for up to 6 months after date of receipt. Avoid repeated freeze/thaw cycles.
    包装信息
    数量 50 µg

    merck millipore,默克密理博,MAB3086,Anti-Ryanodine Receptor Antibody, clone RYR.1

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