Anti-CLCN1 antibody - C-terminal (ab189857)
Key features and details
- Rabbit polyclonal to CLCN1 - C-terminal
- Suitable for: WB
- Reacts with: Mouse, Human
- Isotype: IgG
Overview
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Product name
Anti-CLCN1 antibody - C-terminal -
Description
Rabbit polyclonal to CLCN1 - C-terminal -
Host species
Rabbit -
Tested applications
Suitable for: WBmore details -
Species reactivity
Reacts with: Mouse, Human -
Immunogen
Recombinant fragment within Human CLCN1 (C terminal). The exact sequence is proprietary.
Database link: P35523 -
General notes
The Life Science industry has been in the grips of a reproducibility crisis for a number of years. Abcam is leading the way in addressing this with our range of recombinant monoclonal antibodies and knockout edited cell lines for gold-standard validation. Please check that this product meets your needs before purchasing.
If you have any questions, special requirements or concerns, please send us an inquiry and/or contact our Support team ahead of purchase. Recommended alternatives for this product can be found below, along with publications, customer reviews and Q&As
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
pH: 7.30
Preservative: 0.02% Sodium azide
Constituents: 49% PBS, 50% Glycerol -
Concentration information loading...
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Purity
Immunogen affinity purified -
Clonality
Polyclonal -
Isotype
IgG -
Research areas
Associated products
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Compatible Secondaries
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Isotype control
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Positive Controls
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Recombinant Protein
Applications
The Abpromise guarantee
Our Abpromise guarantee covers the use of ab189857 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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WB |
1/500 - 1/2000. Predicted molecular weight: 109 kDa.
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Notes |
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WB
1/500 - 1/2000. Predicted molecular weight: 109 kDa. |
Target
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Function
Voltage-gated chloride channel. Chloride channels have several functions including the regulation of cell volume; membrane potential stabilization, signal transduction and transepithelial transport. -
Tissue specificity
Predominantly expressed in skeletal muscles. -
Involvement in disease
Defects in CLCN1 are the cause of Thomsen disease (THD) [MIM:160800]; also known as autosomal dominant myotonia congenita (MCD). THD is characterized by skeletal muscle stiffness (delayed relaxation), due to membrane hyperexcitability. A variant form of Thomsen disease is myotonia levior that is characterized by milder symptoms, later onset and absence of muscle hypo- and hypertrophy.
Defects in CLCN1 are the cause of autosomal recessive myotonia congenita (MCR) [MIM:255700]; also known as Becker disease. -
Sequence similarities
Belongs to the chloride channel (TC 2.A.49) family. ClC-1/CLCN1 subfamily.
Contains 2 CBS domains. -
Cellular localization
Membrane. - Information by UniProt
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Database links
- Entrez Gene: 1180 Human
- Entrez Gene: 12723 Mouse
- Omim: 118425 Human
- SwissProt: P35523 Human
- SwissProt: Q64347 Mouse
- Unigene: 121483 Human
- Unigene: 386757 Mouse
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Alternative names
- Chloride channel protein 1 antibody
- Chloride channel protein antibody
- Chloride channel protein skeletal muscle antibody
see all
Images
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Anti-CLCN1 antibody - C-terminal (ab189857) + Mouse skeletal muscle extracts at 25 µg
Secondary
HRP Goat Anti-Rabbit IgG (H+L) at 1/10000 dilution
Predicted band size: 109 kDa
Exposure time: 180 secondsBlocking buffer: 3% nonfat dry milk in TBST.
Detection: ECL Enhanced Kit.
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All lanes : Anti-CLCN1 antibody - C-terminal (ab189857) at 1/500 dilution
Lane 1 : 293T cell extract
Lane 2 : Mouse liver tissue extract
Predicted band size: 109 kDa
Datasheets and documents
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SDS download
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Datasheet download
References (4)
ab189857 has been referenced in 4 publications.
- Mohr M et al. Skeletal muscle phenotype and game performance in elite women football players. Scand J Med Sci Sports N/A:N/A (2021). PubMed: 34427373
- Subramaniam D et al. Suppressing STAT5 signaling affects osteosarcoma growth and stemness. Cell Death Dis 11:149 (2020). PubMed: 32094348
- Altamura C et al. Pathomechanisms of a CLCN1 Mutation Found in a Russian Family Suffering From Becker's Myotonia. Front Neurol 11:1019 (2020). PubMed: 33013670
- Thomassen M et al. Abundance of ClC-1 chloride channel in human skeletal muscle: fiber type specific differences and effect of training. J Appl Physiol (1985) 125:470-478 (2018). PubMed: 29722626