R2029
Monoclonal Anti-Rapsyn antibody produced in mouse
1 mg/mL, clone 1234, purified immunoglobulin, buffered aqueous solution
Synonym(s):
Anti-CMS11, Anti-CMS4C, Anti-FADS, Anti-FADS2, Anti-RAPSYN, Anti-RNF205
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About This Item
Recommended Products
biological source
mouse
Quality Level
conjugate
unconjugated
antibody form
purified immunoglobulin
antibody product type
primary antibodies
clone
1234, monoclonal
form
buffered aqueous solution
species reactivity
amphibian, chicken, mouse, rat, fish
concentration
1 mg/mL
technique(s)
western blot: 0.5 μg/mL using Torpedo californica electrocyte cell extracts
isotype
IgG1
UniProt accession no.
shipped in
dry ice
storage temp.
−20°C
target post-translational modification
unmodified
Gene Information
mouse ... Rapsn(19400)
rat ... Rapsn(362161)
General description
The receptor-associated protein of the synapse (RAPSYN) is a peripheral membrane protein located in the skeletal muscles. It is co-localized along with nicotinic acetylcholine receptors (nAChRs) at the postsynaptic membrane due to the influence of neural agrin. Rapsyn consists of a myristoylated N-terminal, seven putative tetratricopeptide repeats, a coiled-coil domain, the RING-H2 domain, and the consensus sequence for phosphorylation.
Immunogen
whole purified rapsyn from Torpedo californica electric organ postsynaptic membrane.
Application
Monoclonal Anti-Rapsyn antibody produced in mouse has been used in western blotting (0.5 μg/ml ) and immunocytochemistry (1:200).
Biochem/physiol Actions
The receptor-associated protein of the synapse (RAPSYN) participates in assembling the structure of the nicotinic postsynaptic membrane. It is required for the functioning of the neuromuscular junction. Mutations in the RAPSN gene are associated with endplate acetylcholine-receptor deficiency and Myasthenic syndrome.
Physical form
Solution in phosphate buffered saline containing 1.0 mg/mL BSA and 0.05% sodium azide.
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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Storage Class
10 - Combustible liquids
wgk_germany
WGK 3
flash_point_f
Not applicable
flash_point_c
Not applicable
ppe
Eyeshields, Gloves, multi-purpose combination respirator cartridge (US)
Certificates of Analysis (COA)
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Rapsyn mutations in humans cause endplate acetylcholine-receptor deficiency and myasthenic syndrome.
Kinji Ohno et al.
American journal of human genetics, 70(4), 875-885 (2002-01-16)
Congenital myasthenic syndromes (CMSs) stem from genetic defects in endplate (EP)-specific presynaptic, synaptic, and postsynaptic proteins. The postsynaptic CMSs identified to date stem from a deficiency or kinetic abnormality of the acetylcholine receptor (AChR). All CMSs with a kinetic abnormality
M K Ramarao et al.
The Journal of biological chemistry, 276(10), 7475-7483 (2000-11-23)
Rapsyn, a 43-kDa peripheral membrane protein of skeletal muscle, is essential for clustering nicotinic acetylcholine receptors (nAChR) in the postsynaptic membrane. Previous studies with rapsyn NH(2)-terminal fragments fused to green fluorescent protein, expressed in 293T cells along with nAChRs, establish
M Bartoli et al.
The Journal of biological chemistry, 276(27), 24911-24917 (2001-05-09)
Rapsyn, a peripheral membrane protein of skeletal muscle, is necessary for the formation of the highly organized structure of the vertebrate neuromuscular junction. For mice lacking rapsyn, there is a failure of postsynaptic specialization characterized by an absence of nicotinic
Marianna Rodova et al.
Molecular and cellular biology, 24(16), 7188-7196 (2004-07-30)
Rapsyn is a synapse-specific protein that is required for clustering acetylcholine receptors at the neuromuscular junction. Analysis of the rapsyn promoter revealed a consensus site for the transcription factor Kaiso within a region that is mutated in a subset of
Benoît Zuber et al.
Proceedings of the National Academy of Sciences of the United States of America, 110(26), 10622-10627 (2013-06-12)
The scaffolding protein at the neuromuscular junction, rapsyn, enables clustering of nicotinic acetylcholine receptors in high concentration and is critical for muscle function. Patients with insufficient receptor clustering suffer from muscle weakness. However, the detailed organization of the receptor-rapsyn network
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