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MAB3086 Anti-Ryanodine Receptor Antibody, clone RYR.1

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MAB3086
50 µg  
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      Overview

      Key Spec Table

      Species ReactivityKey ApplicationsHostFormatAntibody Type
      B, Ca, H, M, Po, RELISA, FC, ICC, IHC, IP, RIA, WBMPurifiedMonoclonal Antibody
      Description
      Catalogue NumberMAB3086
      Brand Family Chemicon®
      Trade Name
      • Chemicon
      DescriptionAnti-Ryanodine Receptor Antibody, clone RYR.1
      Product Information
      FormatPurified
      PresentationPurified immunoglobulin from culture supernatant. In 10 mM sodium phosphate, 150 mM NaCl, 0.01% NaN3, pH. 7.5
      Quality LevelMQ100
      Applications
      ApplicationAnti-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.
      Key Applications
      • ELISA
      • Flow Cytometry
      • Immunocytochemistry
      • Immunohistochemistry
      • Immunoprecipitation
      • Radioimmunoassay
      • Western Blotting
      Application NotesImmunoblot: 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.
      Biological Information
      ImmunogenShort synthetic polypeptide corresponding to the c-terminal domain of the ryanodine receptor.
      CloneRYR.1
      ConcentrationPlease refer to the Certificate of Analysis for the lot-specific concentration.
      HostMouse
      SpecificityReacts 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).
      IsotypeIgG2b
      Species Reactivity
      • Bovine
      • Canine
      • Human
      • Mouse
      • Pig
      • Rat
      Antibody TypeMonoclonal Antibody
      Entrez Gene Number
      Entrez Gene SummaryThis 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.
      Gene Symbol
      • RYR1
      • CCO
      • RyR1
      • SKRR
      • RYR
      • MHS1
      • MHS
      • RYDR
      • CCD
      • RYR-1
      UniProt Number
      UniProt SummaryFUNCTION: 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.
      Product Usage Statements
      Usage Statement
      • 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.
      Storage and Shipping Information
      Storage ConditionsMaintain at -20°C in undiluted aliquots for up to 6 months after date of receipt. Avoid repeated freeze/thaw cycles.
      Packaging Information
      Material Size50 µg
      Global Trade Item Number
      Catalogue Number GTIN
      MAB3086 04053252504648