Recombinant Anti-Nicotinic Acetylcholine Receptor alpha 1/CHRNA1 antibody [mAb 192] (ab288434)
Key features and details
- Produced recombinantly (animal-free) for high batch-to-batch consistency and long term security of supply
- Rat monoclonal [mAb 192] to Nicotinic Acetylcholine Receptor alpha 1/CHRNA1
- Suitable for: ICC/IF, WB
- Reacts with: Mouse, Human
Overview
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Product name
Anti-Nicotinic Acetylcholine Receptor alpha 1/CHRNA1 antibody [mAb 192]
See all Nicotinic Acetylcholine Receptor alpha 1/CHRNA1 primary antibodies -
Description
Rat monoclonal [mAb 192] to Nicotinic Acetylcholine Receptor alpha 1/CHRNA1 -
Host species
Rat -
Specificity
The antibody binds to human muscle AChR with a Kd of 10 pM, to mouse muscle AChR with a Kd of 50 pM, and to rat muscle AChR with a Kd of 646 nM
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Tested applications
Suitable for: ICC/IF, WBmore details -
Species reactivity
Reacts with: Mouse, Human -
Immunogen
Recombinant full length protein.
Database link: P02708 -
Positive control
- WB: Mouse skeletal muscle lysate ICC/IF: HeLa cells
Properties
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Form
Liquid -
Storage instructions
Shipped at 4°C. Store at +4°C short term (1-2 weeks). Upon delivery aliquot. Store at -20°C long term. Avoid freeze / thaw cycle. -
Storage buffer
Preservative: 0.02% Proclin 300
Constituent: 99% PBS -
Concentration information loading...
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Purity
Protein A purified -
Clonality
Monoclonal -
Clone number
mAb 192 -
Isotype
IgG2b -
Light chain type
kappa -
Research areas
Applications
The Abpromise guarantee
Our Abpromise guarantee covers the use of ab288434 in the following tested applications.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
Application | Abreviews | Notes |
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ICC/IF |
Use a concentration of 10 µg/ml.
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WB |
Use a concentration of 0.03 µg/ml. Predicted molecular weight: 54 kDa.
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Notes |
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ICC/IF
Use a concentration of 10 µg/ml. |
WB
Use a concentration of 0.03 µg/ml. Predicted molecular weight: 54 kDa. |
Target
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Function
After binding acetylcholine, the AChR responds by an extensive change in conformation that affects all subunits and leads to opening of an ion-conducting channel across the plasma membrane. -
Tissue specificity
Isoform 1 is only expressed in skeletal muscle. Isoform 2 is constitutively expressed in skeletal muscle, brain, heart, kidney, liver, lung and thymus. -
Involvement in disease
Defects in CHRNA1 are a cause of multiple pterygium syndrome lethal type (MUPSL) [MIM:253290]. Multiple pterygia are found infrequently in children with arthrogryposis and in fetuses with fetal akinesia syndrome. In lethal multiple pterygium syndrome there is intrauterine growth retardation, multiple pterygia, and flexion contractures causing severe arthrogryposis and fetal akinesia. Subcutaneous edema can be severe, causing fetal hydrops with cystic hygroma and lung hypoplasia. Oligohydramnios and facial anomalies are frequent.
Note=The alpha subunit is the main focus for antibody binding in myasthenia gravis. Myasthenia gravis is characterized by sporadic muscular fatigability and weakness, occurring chiefly in muscles innervated by cranial nerves, and characteristically improved by cholinesterase-inhibiting drugs.
Defects in CHRNA1 are a cause of congenital myasthenic syndrome slow-channel type (SCCMS) [MIM:601462]. SCCMS is the most common congenital myasthenic syndrome. Congenital myasthenic syndromes are characterized by muscle weakness affecting the axial and limb muscles (with hypotonia in early-onset forms), the ocular muscles (leading to ptosis and ophthalmoplegia), and the facial and bulbar musculature (affecting sucking and swallowing, and leading to dysphonia). The symptoms fluctuate and worsen with physical effort. SCCMS is caused by kinetic abnormalities of the AChR, resulting in prolonged endplate currents and prolonged AChR channel opening episodes.
Defects in CHRNA1 are a cause of congenital myasthenic syndrome fast-channel type (FCCMS) [MIM:608930]. FCCMS is a congenital myasthenic syndrome characterized by kinetic abnormalities of the AChR. In most cases, FCCMS is due to mutations that decrease activity of the AChR by slowing the rate of opening of the receptor channel, speeding the rate of closure of the channel, or decreasing the number of openings of the channel during ACh occupancy. The result is failure to achieve threshold depolarization of the endplate and consequent failure to fire an action potential. -
Sequence similarities
Belongs to the ligand-gated ion channel (TC 1.A.9) family. Acetylcholine receptor (TC 1.A.9.1) subfamily. Alpha-1/CHRNA1 sub-subfamily. -
Cellular localization
Cell junction > synapse > postsynaptic cell membrane. Cell membrane. - Information by UniProt
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Database links
- Entrez Gene: 1134 Human
- Entrez Gene: 11435 Mouse
- Omim: 100690 Human
- SwissProt: P02708 Human
- SwissProt: P04756 Mouse
- Unigene: 434479 Human
- Unigene: 4583 Mouse
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Alternative names
- Acetylcholine receptor subunit alpha antibody
- ACHA_HUMAN antibody
- AChR antibody
see all
Images
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Anti-Nicotinic Acetylcholine Receptor alpha 1/CHRNA1 antibody [mAb 192] (ab288434) at 0.03 µg/ml + Mouse Skeletal Muscle lysate at 35 µg
Secondary
anti-mouse IgG1 secondary antibody
Predicted band size: 54 kDa
Observed band size: 58.9 kDa why is the actual band size different from the predicted?The image data was generated using the chimeric mouse version of the same antibody clone
Run on a 10% SDS PAGE gel.
ab288434 successfully detected AChR in mouse skeletal muscle tissue lysate. Though this antibody is targetted to the human AChR it also reacts with mouse AChR.
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The image data was generated using the chimeric mouse version of the same antibody clone
Immunofluorescence analysis of paraformaldehyde fixed HeLa cells, stained for muscle acetylcholine receptor using ab288434 at 10µg/ml for 1 hr. Followed by Alexa Fluor® 488 secondary antibody (2 µg/ml), showing positive cytoplasmic staining.
The nuclear stain is DAPI (blue). Panels show from left-right, top-bottom ab288434, DAPI, merged channels and an isotype control. The isotype control was stained with an unknown specificity antibody (ab288434) followed by Alexa Fluor® 488 secondary antibody.
Protocols
To our knowledge, customised protocols are not required for this product. Please try the standard protocols listed below and let us know how you get on.
Datasheets and documents
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SDS download
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Datasheet download
References (1)
ab288434 has been referenced in 1 publication.
- Kontou M et al. Characterisation, crystallisation and preliminary X-ray diffraction analysis of a Fab fragment of a rat monoclonal antibody with very high affinity for the human muscle acetylcholine receptor. FEBS Lett 389:195-8 (1996). PubMed: 8766828