Product nameAnti-Ryanodine receptor 1 antibody
DescriptionRabbit polyclonal to Ryanodine receptor 1
Tested applicationsSuitable for: ICC/IF, IHC-Pmore details
Species reactivityReacts with: Human
Predicted to work with: Mouse, Rat, Rabbit, Pig
- IHC-P: Human cerebellum tissue. ICC/IF: SiHa cells.
Storage instructionsShipped at 4°C. Store at +4°C short term (1-2 weeks). Upon delivery aliquot. Store at -20°C long term. Avoid freeze / thaw cycle.
Storage bufferpH: 7.20
Preservative: 0.02% Sodium azide
Constituents: 40% Glycerol, PBS
Concentration information loading...
PurityImmunogen affinity purified
Our Abpromise guarantee covers the use of ab224582 in the following tested applications.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
|ICC/IF||Use a concentration of 1 - 4 µg/ml.
Fixation/Permeabilization: PFA/Triton X-100.
|IHC-P||1/20 - 1/50. Perform heat mediated antigen retrieval with citrate buffer pH 6 before commencing with IHC staining protocol.|
FunctionCalcium channel that mediates the release of Ca(2+) from the sarcoplasmic reticulum into the cytoplasm and thereby plays a key role in triggering muscle contraction following depolarization of T-tubules. Repeated very high-level exercise increases the open probability of the channel and leads to Ca(2+) leaking into the cytoplasm. Can also mediate the release of Ca(2+) from intracellular stores in neurons, and may thereby promote prolonged Ca(2+) signaling in the brain. Required for normal embryonic development of muscle fibers and skeletal muscle. Required for normal heart morphogenesis, skin development and ossification during embryogenesis.
Tissue specificitySkeletal muscle and brain (cerebellum and hippocampus).
Involvement in diseaseMalignant hyperthermia 1
Central core disease of muscle
Multiminicore disease with external ophthalmoplegia
Defects in RYR1 may be a cause of Samaritan myopathy, a congenital myopathy with benign course. Patients display severe hypotonia and respiratory distress at birth. Unlike other congenital myopathies, the health status constantly improves and patients are minimally affected at adulthood.
Sequence similaritiesBelongs to the ryanodine receptor (TC 1.A.3.1) family. RYR1 subfamily.
Contains 3 B30.2/SPRY domains.
Contains 5 MIR domains.
DomainThe 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 sarcoplasmic reticulum (SR) and the T-tubule.
modificationsChannel activity is modulated by phosphorylation. Phosphorylation at Ser-2843 may increase channel activity. Repeated very high-level exercise increases phosphorylation at Ser-2843.
Activated by reversible S-nitrosylation. Repeated very high-level exercise increases S-nitrosylation.
Cellular localizationSarcoplasmic reticulum membrane. Membrane. Sarcoplasmic reticulum. The number of predicted transmembrane domains varies between orthologs, but both N-terminus and C-terminus seem to be cytoplasmic.
- Information by UniProt
- CCO antibody
- Central core disease of muscle antibody
- MHS antibody
ab224582 has not yet been referenced specifically in any publications.