货号：05-269

品牌：Merck Millipore

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目录价：￥4505.00

市场价格：￥3829.25

会员价格：￥3604.00

金山科研平台，产品价格货期咨询微信：jinshanbio Description: Anti-Ryanodine Receptor Antibody, clone XA7B6, skeletal muscle | 05-269 View All» Promotional Text: Special Shipping Offer on Antibodies100% Performance Guaranteed View All» Trade Name: Upstate (Millipore) View All» Specificity: skeletal muscle ryanodine receptor Ca++ channel View All» Molecular Weight: 450kDa View All» Immunogen: Rabbit skeletal muscle triads View All» Clone: XA7B6 View All» Isotype: IgM View All» Species Reactivity:

• Canine

• Guinea Pig

• Rat

• Rabbit
  View All» Quality Assurance: routinely evaluated on unboiled triad preparation obtained from rabbit skeletal muscle View All» Purification Method: Ascites View All» Presentation: mouse ascites fluid containing 0.05% sodium azide View All» Storage Conditions: 2 years at -20°C View All» UniProt Number: P21817 View All» Entrez Gene Number: NM_000540 View All» Gene Symbol:
  • RYR1

  • CCO

  • RYR

  • RYR-1

  • SKRR

  • CCD

  • RyR1

  • MHS1

  • MHS

  • RYDR
    View All» 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. View All» Key Applications:
    • Immunohistochemistry

    • Immunoprecipitation

    • Western Blotting
      View All» Entrez Gene Summary: 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. View All» UniProt Summary: 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. View All» Brand Family: Upstate View All» Product Name: Anti-Ryanodine Receptor Antibody, clone XA7B6, skeletal muscle | 05-269 View All» Antibody Type: Monoclonal Antibody View All» Qty/Pk: 100 µL View All» Format: Ascites View All» Host: Mouse View All»
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