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产品信息
PA5-37147
应用
建议稀释比
已发表文章
产品规格
种属反应
Human,
Mouse,
Rat
已发表种属
Human
宿主/亚型
Rabbit
/ IgG
分类
Polyclonal
类型
Antibody
抗原
A synthetic peptide corresponding to residues in human CLCN1
偶联物
形式
Liquid
浓度
1 mg/mL
纯化类型
Antigen affinity chromatography
保存液
PBS with 50% glycerol
内含物
0.02% sodium azide
保存条件
Store at 4°C short term. For long term storage, store at -20°C, avoiding freeze/thaw cycles.
运输条件
Wet ice
RRID
AB_2553930
产品详细信息
This antibody detects endogenous protein at a molecular weight of 110 kDa.
Purity is >95% by SDS-PAGE.
靶标信息
The family of voltage-dependent chloride channels (CLCs) regulate cellular trafficking of chloride ions, a critical component of all living cells. CLCs regulate excitability in muscle and nerve cells, aid in organic solute transport and maintain cellular volume. CLC1 is highly expressed in skeletal muscle. Mutations in the gene encoding CLC1 lead to myotonia, an inheritable disorder characterized by muscle stiffness and renal salt wasting. CLC2 is highly expressed in the epithelia of several organs including lung, which suggests CLC2 may be a possible therapeutic target for cystic fibrosis. CLC3 expression is particularly abundant in neuronal tissue, while CLC4 expression is evident in skeletal and cardiac muscle as well as brain. Mutations in the gene encoding CLC5 lead to Dent's disease, a renal disorder characterized by proteinuria and hypercalciuria. CLC6 and CLC7 are broadly expressed in several tissues including testis, kidney, brain and muscle.
仅用于科研。不用于诊断过程。未经明确授权不得转售。
生物信息学
蛋白别名: arrested development of righting response; chloride channel 1, skeletal muscle; Chloride channel protein 1; Chloride channel protein, skeletal muscle; chloride channel, voltage-sensitive 1; CIC 1; CIC1; ClC-1; MGC138361; MGC142055
基因别名: adr; Clc-1; CLC1; CLCN1; mto; myotonia; nmf355; SMCC; SMCC1
UniProt ID: (Human) P35523, (Mouse) Q64347, (Rat) P35524
Entrez Gene ID: (Human) 1180, (Mouse) 12723, (Rat) 25688
muscle contraction
neuronal action potential propagation
ion transmembrane transport
chloride transmembrane transport
regulation of anion transmembrane transport
transport
ion transport
chloride transport
regulation of ion transmembrane transport
transmembrane transport
regulation of anion transport
