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R2029

Sigma-Aldrich

Monoclonal Anti-Rapsyn antibody produced in mouse

1 mg/mL, clone 1234, purified immunoglobulin, buffered aqueous solution

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Synonym(s):
Anti-CMS11, Anti-CMS4C, Anti-FADS, Anti-FADS2, Anti-RAPSYN, Anti-RNF205
MDL number:
NACRES:
NA.41

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.

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)


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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
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
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
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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