抗体

兔抗人类氯离子通道1, 骨骼肌多克隆抗体

MBS714620

0.05毫升

375美元

多克隆

兔

未共轭

人类, 大鼠

免疫印迹, 酶联免疫吸附测定

抗体

兔抗人类氯离子通道1, 骨骼肌多克隆抗体

氯离子通道1, 骨骼肌

氯离子通道1;骨骼肌;CLCN1;CLC1;MGC138361;MGC142055

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

CLCN1; CLCN1; CLC1; CLC1; ClC-1

CLCN1_HUMAN

多克隆

IgG

兔

Human; Other species are not tested. Please decide the specificity by homology.

988

抗原亲和纯化

液体

Shipped at 4 degree C. Upon delivery aliquot and store at -20 degree C or -80 degree C. Avoid repeated freeze.

酶联免疫吸附测定;尚未在其他应用中测试

Immunogen: Human CLCN1. Conjugate: Non-conjugated

Storage Buffer: PBS with 0.02% sodium azide and 50% glycerol pH 7.3.

Voltage-gated chloride channel. Chloride channels have several functions including the regulation of cell volume; membrane potential stabilization, signal transduction and transepithelial transport .

119433677

NP_000074.2

NM_000083.2

Ion Channel Transport Pathway (187193); Stimuli-sensing Channels Pathway (771568); Transmembrane Transport Of Small Molecules Pathway (106572)

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]

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

0.05毫升

375美元

0.15毫升

715