product summary
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company name :
MyBioSource
product type :
antibody
product name :
Rabbit anti-human chloride channel 1, skeletal muscle polyclonal Antibody
catalog :
MBS714620
quantity :
0.05 mL
price :
375 USD
clonality :
polyclonal
host :
rabbit
conjugate :
nonconjugated
reactivity :
human, rat
application :
western blot, ELISA
more info or order :
MyBioSource product webpage
citations: 2
Published Application/Species/Sample/DilutionReference
  • western blot; rat; 1:200; loading ...; fig 3a
Camerino G, De Bellis M, Conte E, Liantonio A, Musaraj K, Cannone M, et al. Statin-induced myotoxicity is exacerbated by aging: A biophysical and molecular biology study in rats treated with atorvastatin. Toxicol Appl Pharmacol. 2016;306:36-46 pubmed publisher
  • western blot; human; fig 3
Imbrici P, Altamura C, Camerino G, Mangiatordi G, Conte E, Maggi L, et al. Multidisciplinary study of a new ClC-1 mutation causing myotonia congenita: a paradigm to understand and treat ion channelopathies. FASEB J. 2016;30:3285-3295 pubmed
product information
catalog number :
MBS714620
products type :
Antibody
products full name :
Rabbit anti-human chloride channel 1, skeletal muscle polyclonal Antibody
products short name :
chloride channel 1, skeletal muscle
products name syn :
chloride channel 1; skeletal muscle; CLCN1; CLC1; MGC138361; MGC142055
other names :
chloride channel protein 1; Chloride channel protein 1; chloride channel protein 1; clC-1; chloride channel 1, skeletal muscle; chloride channel protein, skeletal muscle; chloride channel, voltage-sensitive 1; Chloride channel protein, skeletal muscle
other gene names :
CLCN1; CLCN1; CLC1; CLC1; ClC-1
uniprot entry name :
CLCN1_HUMAN
clonality :
Polyclonal
isotype :
IgG
host :
Rabbit
reactivity :
Human; Other species are not tested. Please decide the specificity by homology.
sequence length :
988
purity :
Antigen Affinity Purified
form :
Liquid
storage stability :
Shipped at 4 degree C. Upon delivery aliquot and store at -20 degree C or -80 degree C. Avoid repeated freeze.
tested application :
ELISA;Not yet tested in other applications.
other info1 :
Immunogen: Human CLCN1. Conjugate: Non-conjugated
other info2 :
Storage Buffer: PBS with 0.02% sodium azide and 50% glycerol pH 7.3.
products description :
Voltage-gated chloride channel. Chloride channels have several functions including the regulation of cell volume; membrane potential stabilization, signal transduction and transepithelial transport .
ncbi gi num :
119433677
ncbi acc num :
NP_000074.2
ncbi gb acc num :
NM_000083.2
ncbi pathways :
Ion Channel Transport Pathway (187193); Stimuli-sensing Channels Pathway (771568); Transmembrane Transport Of Small Molecules Pathway (106572)
ncbi summary :
The CLCN family of voltage-dependent chloride channel genes comprises nine members (CLCN1-7, Ka and Kb) which demonstrate quite diverse functional characteristics while sharing significant sequence homology. The protein encoded by this gene regulates the electric excitability of the skeletal muscle membrane. Mutations in this gene cause two forms of inherited human muscle disorders: recessive generalized myotonia congenita (Becker) and dominant myotonia (Thomsen). Alternative splicing results in multiple transcript variants. [provided by RefSeq, Mar 2012]
uniprot summary :
CLCN1: Voltage-gated chloride channel. Chloride channels have several functions including the regulation of cell volume; membrane potential stabilization, signal transduction and transepithelial transport. Defects in CLCN1 are the cause of Thomsen disease (THD); 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); also known as Becker disease. Belongs to the chloride channel (TC 2.A.49) family. ClC-1/CLCN1 subfamily. Protein type: Transporter; Channel, chloride; Transporter, ion channel; Membrane protein, integral; Membrane protein, multi-pass. Chromosomal Location of Human Ortholog: 7q35. Cellular Component: integral to plasma membrane; plasma membrane; sarcolemma. Molecular Function: chloride channel activity; voltage-gated chloride channel activity. Biological Process: muscle contraction; transport; action potential propagation; transmembrane transport. Disease: Myotonia Congenita, Autosomal Recessive; Myotonia Congenita, Autosomal Dominant
size1 :
0.05 mL
price1 :
375 USD
size2 :
0.15 mL
price2 :
715
more info or order :
MyBioSource product webpage
company information
MyBioSource
P.O. Box 153308
San Diego, CA 92195-3308
sales@mybiosource.com
https://www.mybiosource.com
1-888-627-0165
headquarters: USA
MyBioSource, LLC was orginally founded in Vancouver by three enthusiastic scientists who are passionate about providing the world with the best reagents available. Together, they form a company with a big vision known as MyBioSource. MyBioSource is now located in San Diego, California, USA.

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