RAPSN rabbit pAb

RAPSN rabbit pAb

AO-06-ES13462-50

RAPSN rabbit pAb 50μL

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€299.00
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Antibody Product Overview

ELK.NoES13462
Product nameRAPSN rabbit pAb
ReactivityHuman; Mouse
ApplicationsWB;IHC
Other name
Size50μL
Unit price ($)148
Human gene ID5913
Human Swiss-ProtQ13702
SourceRabbit
IsotypeIgG
TargetRAPSN
Fields
Gene nameRAPSN RNF205
Protein nameRAPSN
Human gene linkView Human Gene
Human Swiss linkView Human Swiss-Prot
Mouse gene ID19400
Mouse gene linkView Mouse Gene
Mouse Swiss-ProtP12672
Mouse Swiss linkView Mouse Swiss-Prot
Rat gene ID
Rat gene link
Rat Swiss-Prot
Rat Swiss link
ImmunogenSynthesized peptide derived from human RAPSN AA range: 341-391
SpecificityThis antibody detects endogenous levels of RAPSN at Human/Mouse
FormulationLiquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
ClonalityPolyclonal
DilutionWB 1:500-2000;IHC-p 1:50-300
PurificationThe antibody was affinity-purified from rabbit serum by affinity-chromatography using specific immunogen.
Concentration1 mg/ml
Storage stability-20°C/1 year
Molecular Weight (Da)45kD
Observed band (KD)
BackgroundThis gene encodes a member of a family of proteins that are receptor associated proteins of the synapse. The encoded protein contains a conserved cAMP-dependent protein kinase phosphorylation site, and plays a critical role in clustering and anchoring nicotinic acetylcholine receptors at synaptic sites by linking the receptors to the underlying postsynaptic cytoskeleton, possibly by direct association with actin or spectrin. Mutations in this gene may play a role in postsynaptic congenital myasthenic syndromes. Alternatively spliced transcript variants encoding multiple isoforms have been observed for this gene. [provided by RefSeq, Apr 2011],
Functiondisease:Defects in RAPSN are a cause of congenital myasthenic syndrome type 1d (CMS1D) [MIM:608931]; also called congenital myasthenic syndrome associated with acetylcholine receptor deficiency. Congenital myasthenic syndromes (CMS) are inherited disorders of neuromuscular transmission that stem from mutations in presynaptic, synaptic, or postsynaptic proteins. Postsynaptic disorders result from mutations in proteins forming the subunits of the muscle acetylcholine receptor (AChR). The kinetic abnormalities of AChR result in either prolonged ion channel activations that underlie "slow-channel myasthenic syndromes" (SCCMS) or abbreviated channel activations that underlie the abnormally rapid decay of endplate currents in "fast-channel syndromes" (FCCMS). CMS1D is the third disorder associated with postsynaptic CMS which could result from mutations in the proteins forming the muscle AChR.
Subcellular locationCell membrane; Peripheral membrane protein; Cytoplasmic side. Cell junction, synapse, postsynaptic cell membrane; Peripheral membrane protein; Cytoplasmic side. Cytoplasm, cytoskeleton. Cytoplasmic surface of postsynaptic membranes.
Expression

Additional Images

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Western blot analysis of lysates from U2OS cells, primary antibody was diluted at 1:1000, 4°over night
Image 2
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Immunohistochemical analysis of paraffin-embedded human Colon cancer. 1, Antibody was diluted at 1:200(4° overnight). 2, Tris-EDTA,pH9.0 was used for antigen retrieval. 3,Secondary antibody was diluted at 1:200(room temperature, 45min).
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: AO-06-ES13462-50
: 10 Items
Hurry! only 10 items left in stock.

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