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About This Item
biological source
rabbit
Quality Level
recombinant
expressed in HEK 293 cells
conjugate
unconjugated
antibody form
purified antibody
antibody product type
primary antibodies
clone
1K4, recombinant monoclonal
description
recombinant, expressed in HEK 293 cells
product line
ZooMAb® learn more
form
lyophilized
mol wt
calculated mol wt 565.18 kDa, observed mol wt ~565 kDa
purified by
using Protein A
species reactivity
mouse, human
packaging
antibody small pack of 25
enhanced validation
recombinant expression
Learn more about Antibody Enhanced Validation
technique(s)
affinity binding assay: suitable, immunocytochemistry: suitable, immunohistochemistry: suitable, western blot: suitable
isotype
IgG
epitope sequence
N-terminal cytoplasmic domain
Protein ID accession no.
UniProt accession no.
shipped in
ambient
storage temp.
2-8°C
Gene Information
human ... RYR1(6261)
General description
Ryanodine receptor 1 (UniProt: P21817; also known as RYR-1, RyR1, Skeletal muscle calcium release channel, Skeletal muscle ryanodine receptor, Skeletal muscle-type ryanodine receptor, Type 1 ryanodine receptor) is encoded by the RYR1 (also known as RYDR) gene (Gene ID: 6261) in human. The ryanodine receptor (RyR) is the channel responsible for the release of Ca2+ from the sarcoplasmic reticulum (SR) in muscle cells and it also plays a role in Ca2+ regulation in non-muscle cells. In human, RyR1 plays an important role in muscle contraction. RYR1 is a multi-pass membrane protein and exists as a homotetrameric protein. Structurally, RYR1 is comprised of approximately 5,038 amino acids; has a big N-terminal cytoplasmic domain, ~6 transmembrane domains and a short C-terminus cytoplasmic domain. RYR1 is predominantly expressed in skeletal muscle, brain, and notable expression in cardiac muscle as well. Subcellularly, it is localized to the sarcoplasmic reticulum membrane where it interacts with various proteins involved in calcium homeostasis, such as triadin and calsequestrin. Three isoforms of RYR1 have been described that are produced by alternative splicing. Mutations in the RYR1 gene are implicated in several myopathies, including malignant hyperthermia susceptibility (MHS), central core disease (CCD), and King-Denborough syndrome, leading to significant clinical manifestations such as muscle weakness, respiratory failure, and increased susceptibility to anesthetic agents. The phenotypic spectrum of RYR1-related disorders is vast, with mutations often correlating with disease severity and specific clinical features. Recent studies have highlighted the functional consequences of RYR1 mutations on calcium channel activity, which can disrupt normal muscle function and contribute to the pathophysiology of these diseases. This ZooMAb® recombinant monoclonal antibody, generated by our proprietary technology, offers significantly enhanced specificity, affinity, reproducibility, and stability over conventional monoclonals. (Ref.: Witherspoon, J.W., et al. (2016). Acta Neuropathol. Commun. 4; 121; Cacheux, M., et al. (2015). J Neuromuscul Dis. 2(4); 421-432).
Immunogen
Application
Evaluated by Western Blotting in human skeletal muscle tissue lysate.
Western Blotting Analysis: A 1:10,000 dilution of this antibody detected RYR1 in human skeletal muscle tissue lysate.
Tested Applications
Immunocytochemistry Analysis: A 1:100 dilution from a representative lot detected RYR1 in C2C12 cells.
Affinity Binding Assay: A representative lot of this antibody bound RYR1 peptide with at least one hundred thousand-fold (100,000X) higher affinity than with non-specific control peptide.
Immunohistochemistry (Paraffin) Analysis: A 1:1,000 dilution from a representative lot detected RYR1 in human cerebellum tissue sections.
Note: Actual optimal working dilutions must be determined by end user as specimens, and experimental conditions may vary with the end user.
Biochem/physiol Actions
Physical form
Preparation Note
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Storage Class
11 - Combustible Solids
flash_point_f
Not applicable
flash_point_c
Not applicable
Regulatory Information
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Global Trade Item Number
| SKU | GTIN |
|---|---|
| ZRB2525-4X25UL | 04065273706772 |
| ZRB2525-25UL | 04065273706765 |
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