AN301983L
Recombinant Ryanodine Receptor Monoclonal Antibody
Size
£252.90
Was £281.00
- SKU:
- AN301983L
- Additional Names:
- PPP1R,CCO,KDS,MHS,RYR,MHS1,RYDR,SKRR,RYR-1,PPP1R137,RYR1
- Application:
- IHC
- Concentration:
- 1 mg/ml
- Purification:
- Protein A Purified
- Storage Conditions:
- -20[o]C Avoid freeze/thaw cycles.
- Supplier:
- Elabscience
- Host:
- Rabbit
- Reactivities:
- Human, Mouse, Rat
- Buffer:
- PBS, 50% glycerol, 0.05% Proclin 300, 0.05% protein protectant.
- Immunogen:
- Peptide. This information is proprietary to PTMab.
- Clone:
- A703
- Uniprot:
- P21817
- Synonyms:
- CCO;central core disease of muscle;MHS;MHS1;PPP1R137;protein phosphatase 1, regulatory subunit 137;ryanodine receptor 1;ryanodine receptor 1 (skeletal);RYDR;RYR;RYR-1;sarcoplasmic reticulum calcium release channel;skeletal muscle calcium release channel;skeletal muscle ryanodine receptor;Skeletal muscle-type ryanodine receptor;SKRR;Type 1 ryanodine receptor;type 1-like ryanodine receptor
- Extra Details:
- Ryanodine receptors (RyRs) are large (>500 kDa), intracellular calcium channels found in the sarcoplasmic/endoplasmic reticulum membrane and are responsible for the release of Ca2+ from intracellular stores in excitable cells, such as muscle and neurons. RyRs exist as three mammalian isoforms (RyR1-3), all of which form homotetramers regulated by phosphorylation and/or direct or indirect interaction with a variety of proteins (L-type calcium channels, PKA, FKBP12/12.6, CaMKII, calmodulin, calsequestrin, junctin, and triadin) and ions (Mg2+ and Ca2+). Regulation of the RyR channel by protein modulators occurs within the large cytoplasmic domain, whereas the carboxy terminal portion of the protein forms the ion-binding and conducting pore. RyR1 and RyR2 are predominantly expressed in skeletal and cardiac muscle, respectively, where they localize exclusively to the sarcoplasmic reticulum (SR) and facilitate calcium-mediated communication between transverse-tubules and sarcoplasmic reticulum. Contraction of skeletal muscle is triggered by release of calcium ions from the SR following depolarization of T-tubules. Research studies have shown that defects in RyR1 are the cause of malignant hyperthermia susceptibility type 1 (MHS1), central core disease of muscle (CCD), multiminicore disease with external ophthalmoplegia, and congenital myopathy with fiber-type disproportion (CFTD), each of which is manifested by defects in muscle function, metabolism, and development.
- Shipping Conditions:
- Blue Ice