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
ZooMAb® antibodies represent an entirely new generation of recombinant monoclonal antibodies. Each ZooMAb® antibody is manufactured using our proprietary recombinant expression system, purified to homogeneity, and precisely dispensed to produce robust and highly reproducible lot-to-lot consistency. Only top-performing clones are released for use by researchers. Each antibody is validated for high specificity and affinity across multiple applications, including its most commonly used application. ZooMAb® antibodies are reliably available and ready to ship when you need them.
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).
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
KLH-conjugated linear peptide corresponding to 24 amino acids from the N-terminal cytoplasmic domain of human RYR1.
Application
Quality Control Testing
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.
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
Clone 1K4 is a ZooMAb® recombinant rabbit monoclonal antibody that specifically detects RYR1. It targets an epitope within 24 amino acids from the N-terminal cytoplasmic domain.
Physical form
Purified recombinant rabbit monoclonal antibody IgG, lyophilized in PBS with 5% Trehalose, normal appearance a coarse or translucent resin. The PBS/trehalose components in the ZooMAb formulation can have the appearance of a semi-solid (bead like gel) after lyophilization. This is a normal phenomenon. Please follow the recommended reconstitution procedure in the data sheet to dissolve the semi-solid, bead-like, gel-appearing material. The resulting antibody solution is completely stable and functional as proven by full functional testing. Contains no biocide or preservatives, such as azide, or any animal by-products. Larger pack sizes provided as multiples of 25 µL.
Preparation Note
300 µg/mL after reconstitution at 25 µL per vial. Please refer to guidance on suggested starting dilutions and/or titers per application and sample type.
Recommend storage of lyophilized product at 2-8°C; Before reconstitution, micro-centrifuge vials briefly to spin down material to bottom of the vial; Reconstitute each vial by adding 25 µL of filtered lab grade water or PBS; Reconstituted antibodies can be stored at 2-8°C, or -20°C for long term storage. Avoid repeated freeze-thaws.
Legal Information
ZooMAb is a registered trademark of Merck KGaA, Darmstadt, Germany
Disclaimer
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.
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存储类别
11 - Combustible Solids
flash_point_f
Not applicable
flash_point_c
Not applicable
法规信息
新产品
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全球贸易项目编号
| 货号 | GTIN |
|---|---|
| ZRB2525-4X25UL | 04065273706772 |
| ZRB2525-25UL | 04065273706765 |
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