生物来源
rabbit
偶联物
unconjugated
抗体形式
affinity isolated antibody
抗体产品类型
primary antibodies
克隆
polyclonal
表单
lyophilized powder
种属反应性
mouse, rat
技术
western blot (chemiluminescent): 1:200
UniProt登记号
储存温度
−20°C
基因信息
human ... CLCN1(1180)
mouse ... Clcn1(12723)
rat ... Clcn1(25688)
免疫原
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.
应用
Anti-Chloride Channel-1 (CLC-1) antibody produced in rabbit is suitable for western blotting (chemiluminescent) at a working dilution of 1:200.
生化/生理作用
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).
外形
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.
免责声明
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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储存分类代码
13 - Non Combustible Solids
WGK
WGK 3
闪点(°F)
Not applicable
闪点(°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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