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Key Specifications Table
| Species Reactivity | Key Applications | Host | Format | Antibody Type |
|---|---|---|---|---|
| H, M, R | ICC, IHC, WB | Rb | Serum | Polyclonal Antibody |
| Description | |
|---|---|
| Catalogue Number | AB9078 |
| Brand Family | Chemicon® |
| Trade Name |
|
| Description | Anti-Ryanodine Receptor 1 Antibody |
| Product Information | |
|---|---|
| Format | Serum |
| Control |
|
| Presentation | Rabbit serum. Liquid. |
| Quality Level | MQ100 |
| Applications | |
|---|---|
| Application | Anti-Ryanodine Receptor 1 Antibody detects level of Ryanodine Receptor 1 & has been published & validated for use in IC, IH & WB. |
| Key Applications |
|
| Application Notes | Western blot: 1:1,000 using ECL. The antibody reacts with the 500 kDa Ryanodine Receptor 1 protein. Suggested blocking buffer is 10% normal goat serum (or same host as your secondary antibody), 1% BSA in 0.1M PBS with 0.05% Tween 20. Suggested dilution buffer is 1% normal goat serum (or same host as your secondary antibody), 1% BSA in 0.1M PBS with 0.05% Tween 20. Preferred gel percentage is 4-12% gradient gel. Immunocytochemistry: 1:1,000 Immunohistochemistry: 1:1,000 overnight at 2-8°C using a fluorescently labeled secondary antibody. Suggested fixative is 4% paraformaldehyde in 0.1M PBS (one hour). Suggested permeablization method is 0.05% Triton X-100 in dilution buffer. Suggested blocking buffer is 10% normal goat serum (or same host as your secondary antibody) and 1% BSA in 0.1M PBS. Suggested dilution buffer is 1% normal goat serum (or same host as your secondary antibody) and 1% BSA in 0.1M PBS. Optimal working dilutions must be determined by the end user. |
| Biological Information | |
|---|---|
| Immunogen | Synthetic peptide from the variant TM region of human Ryanodine Receptor 1. |
| Host | Rabbit |
| Specificity | Ryanodine Receptor 1. |
| Species Reactivity |
|
| Antibody Type | Polyclonal Antibody |
| Entrez Gene Number |
|
| 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. |
| Gene Symbol |
|
| UniProt Number |
|
| 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. |
| Product Usage Statements | |
|---|---|
| Usage Statement |
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| Storage and Shipping Information | |
|---|---|
| Storage Conditions | Maintain at -20°C in undiluted aliquots for up to 6 months after date of receipt. Avoid repeated freeze/thaw cycles. |
| Packaging Information | |
|---|---|
| Material Size | 100 µL |