biological source
rabbit
conjugate
unconjugated
antibody form
affinity isolated antibody
antibody product type
primary antibodies
clone
polyclonal
form
lyophilized powder
species reactivity
mouse, rat
technique(s)
western blot (chemiluminescent): 1:200
UniProt accession no.
storage temp.
−20°C
Gene Information
human ... CLCN1(1180)
mouse ... Clcn1(12723)
rat ... Clcn1(25688)
Immunogen
synthetic peptide corresponding to amino acids 102-117 of rat or mouse CLC-1. The epitope is identical in mouse and rat and homologous to human and goat.
Application
Anti-Chloride Channel-1 (CLC-1) antibody produced in rabbit is suitable for western blotting (chemiluminescent) at a working dilution of 1:200.
Biochem/physiol Actions
The protein encoded by this voltage-gated chloride channel gene is involved in regulating the electric excitability of the skeletal muscle membrane. The channel is a homodimer in which each monomer supports an identical pore carrying three anion-binding sites. Mutation in this gene causes two forms of inherited human muscle disorders: recessive generalized myotonia congenita (Becker) and dominant myotonia (Thomsen).
Physical form
Lyophilized at ~0.3 mg/ml from phosphate buffered saline, pH 7.4, containing 1% bovine serum albumin, 5% sucrose, and 0.025% 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.
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存储类别
13 - Non Combustible Solids
wgk
WGK 3
flash_point_f
Not applicable
flash_point_c
Not applicable
法规信息
新产品
此项目有
Jean-François Desaphy et al.
Experimental neurology, 248, 530-540 (2013-08-13)
Myotonia congenita (MC) is caused by loss-of-function mutations of the muscle ClC-1 chloride channel. Clinical manifestations include the variable association of myotonia and transitory weakness. We recently described a cohort of recessive MC patients showing, at a low rate repetitive
Ryogen Sasaki et al.
Rinsho shinkeigaku = Clinical neurology, 53(4), 316-319 (2013-04-23)
Autosomal-dominant type of myotonia (Thomsen's disease) and autosomal-recessive one (Becker's disease) are caused by mutations in the skeletal muscle voltage-gated chloride channel gene (CLCN1). Clinical manifestation of the diseases ranges from minimum to severely disabling myotonia. We report a Japanese
Brett Bennetts et al.
Nature communications, 4, 2507-2507 (2013-09-26)
Uniquely, the ClC family harbours dissipative channels and anion/H(+) transporters that share unprecedented functional characteristics. ClC-1 channels are homodimers in which each monomer supports an identical pore carrying three anion-binding sites. Transient occupancy of the extracellular binding site by a
Randal C Richardson et al.
Muscle & nerve, 49(4), 593-600 (2013-07-31)
Myotonia congenita due to protein truncating CLCN1 mutations is associated with variable patterns of inheritance. Three family kindreds are described, all of whom possess protein truncating mutations (Y33X, fs503X, R894X). One lineage also has coexistent R894X, A313T, and A320V mutations.
M C Koch et al.
Science (New York, N.Y.), 257(5071), 797-800 (1992-08-07)
Autosomal recessive generalized myotonia (Becker's disease) (GM) and autosomal dominant myotonia congenita (Thomsen's disease) (MC) are characterized by skeletal muscle stiffness that is a result of muscle membrane hyperexcitability. For both diseases, alterations in muscle chloride or sodium currents or
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