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merck millipore,默克密理博,05-269,Anti-Ryanodine Receptor Antibody, clone XA7B6, skeletal muscle
产品名称：Anti-Ryanodine Receptor Antibody, clone XA7B6, skeletal muscle
产品型号：05-269
This Anti-Ryanodine Receptor Antibody, clone XA7B6, skeletal muscle is validated for use in IH, IP, WB for the detection of Ryanodine Receptor.
merck millipore,默克密理博,05-269,Anti-Ryanodine Receptor Antibody, clone XA7B6, skeletal muscle
- 产品介绍
重要规格表
品种反应性 主要应用 宿主 格式 抗体类型 Rb, Ca, Gp, R WB, IHC M Ascites Monoclonal Antibody 描述 产品目录编号 05-269 品牌系列 Upstate
商名 - Upstate
描述 Anti-Ryanodine Receptor Antibody, clone XA7B6, skeletal muscle 产品信息 格式 Ascites 演示 mouse ascites fluid containing 0.05% sodium azide 应用 应用 This Anti-Ryanodine Receptor Antibody, clone XA7B6, skeletal muscle is validated for use in IH, IP, WB for the detection of Ryanodine Receptor. 主要应用 - Western Blotting
- Immunohistochemistry
生物信息 免疫原品种 Rabbit skeletal muscle triads 克隆 XA7B6 宿主 Mouse 特异性 skeletal muscle ryanodine receptor Ca++ channel 同种型 IgM 品种反应性 RabbitCanineGuinea PigRat 抗体类型 Monoclonal Antibody Entrez基因编号 - NM_000540
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 - RYDR
- RYR1
- CCO
- RYR
- RYR-1
- SKRR
- CCD
- RyR1
- MHS1
- MHS
纯化方法 Ascites 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 质量保证 routinely evaluated on unboiled triad preparation obtained from rabbit skeletal muscle 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 2 years at -20°C 包装信息 数量 100 µL
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