推荐产品
生物源
rabbit
共軛
unconjugated
抗體表格
affinity isolated antibody
抗體產品種類
primary antibodies
無性繁殖
polyclonal
產品線
Prestige Antibodies® Powered by Atlas Antibodies
形狀
buffered aqueous glycerol solution
物種活性
human
技術
immunofluorescence: 0.25-2 μg/mL
immunohistochemistry: 1:50-1:200
免疫原序列
GVDHAYVTSIGRLIGIVTLKELRKAIEGSVTAQGVKVRPPLASFRDSATSSSDTETTEVHALWGPHSRHG
UniProt登錄號
運輸包裝
wet ice
儲存溫度
−20°C
目標翻譯後修改
unmodified
基因資訊
human ... CLCN2(1181)
一般說明
CLCN2 (chloride channel, voltage-sensitive 2) is a member of the voltage gated Cl- channels and Cl-/H+ exchangers called ClC family of proteins. The mammalian family consists of nine members including CLCN2. This protein has a ubiquitous expression pattern. It has a predominant expression in brain, pancreas, kidney and intestine. This protein is localized intracellulary in the basolateral membrane of polarized cells. It exists as aggregates on the cell surface, and some proportion of it localized with lipid rafts. This protein consists of two putative phosphorylation sites in its N-terminal, and seven in its C-terminal.
免疫原
Chloride channel protein 2 recombinant protein epitope signature tag (PrEST)
應用
All Prestige Antibodies Powered by Atlas Antibodies are developed and validated by the Human Protein Atlas (HPA) project and as a result, are supported by the most extensive characterization in the industry.
The Human Protein Atlas project can be subdivided into three efforts: Human Tissue Atlas, Cancer Atlas, and Human Cell Atlas. The antibodies that have been generated in support of the Tissue and Cancer Atlas projects have been tested by immunohistochemistry against hundreds of normal and disease tissues and through the recent efforts of the Human Cell Atlas project, many have been characterized by immunofluorescence to map the human proteome not only at the tissue level but now at the subcellular level. These images and the collection of this vast data set can be viewed on the Human Protein Atlas (HPA) site by clicking on the Image Gallery link. We also provide Prestige Antibodies® protocols and other useful information.
The Human Protein Atlas project can be subdivided into three efforts: Human Tissue Atlas, Cancer Atlas, and Human Cell Atlas. The antibodies that have been generated in support of the Tissue and Cancer Atlas projects have been tested by immunohistochemistry against hundreds of normal and disease tissues and through the recent efforts of the Human Cell Atlas project, many have been characterized by immunofluorescence to map the human proteome not only at the tissue level but now at the subcellular level. These images and the collection of this vast data set can be viewed on the Human Protein Atlas (HPA) site by clicking on the Image Gallery link. We also provide Prestige Antibodies® protocols and other useful information.
生化／生理作用
CLCN2 (chloride channel, voltage-sensitive 2) has a high level of expression in the brain, where it mediates inhibitory γ-aminobutyric acid (GABA) response. It maintains low intracellular Cl- levels, to achieve this. Hyperpolarization of membrane and intracellular anion concentration activate CLCN2, and it is involved in solute transport across epithelial cells. It is expressed in lung epithelia, where it is capable of Cl- transport. It might also be involved in the morphogenesis of lungs. In astrocytes, this protein is involved in the maintenance of extracellular electrolyte homeostasis. Mutation M200fsX231 produces an extremely truncated CLCN2 protein, and might be linked with idiopathic generalized epilepsy. Studies also suggest that this protein might be associated with atherosclerosis and constipation-associated irritable bowel disease. Studies show that the susceptibility to a subset of childhood absence epilepsies maybe linked to CLCN2 gene locus.
特點和優勢
Prestige Antibodies® are highly characterized and extensively validated antibodies with the added benefit of all available characterization data for each target being accessible via the Human Protein Atlas portal linked just below the product name at the top of this page. The uniqueness and low cross-reactivity of the Prestige Antibodies® to other proteins are due to a thorough selection of antigen regions, affinity purification, and stringent selection. Prestige antigen controls are available for every corresponding Prestige Antibody and can be found in the linkage section.
Every Prestige Antibody is tested in the following ways:
Every Prestige Antibody is tested in the following ways:
- IHC tissue array of 44 normal human tissues and 20 of the most common cancer type tissues.
- Protein array of 364 human recombinant protein fragments.
聯結
Corresponding Antigen APREST72848
外觀
Solution in phosphate-buffered saline, pH 7.2, containing 40% glycerol and 0.02% sodium azide
法律資訊
Prestige Antibodies is a registered trademark of Merck KGaA, Darmstadt, Germany
免責聲明
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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儲存類別代碼
10 - Combustible liquids
水污染物質分類（WGK）
WGK 1
閃點（°F）
Not applicable
閃點（°C）
Not applicable
個人防護裝備
Eyeshields, Gloves, multi-purpose combination respirator cartridge (US)
Ute I Scholl et al.
Nature genetics, 50(3), 349-354 (2018-02-07)
Primary aldosteronism, a common cause of severe hypertension 1 , features constitutive production of the adrenal steroid aldosterone. We analyzed a multiplex family with familial hyperaldosteronism type II (FH-II) 2 and 80 additional probands with unsolved early-onset primary aldosteronism. Eight
Kate Everett et al.
Epilepsy research, 75(2-3), 145-153 (2007-06-21)
In order to assess the chloride channel gene CLCN2 as a candidate susceptibility gene for childhood absence epilepsy, parametric and non-parametric linkage analysis was performed in 65 nuclear pedigrees. This provided suggestive evidence for linkage with heterogeneity: NPL score=2.3, p<0.009;
Carol J Blaisdell et al.
BMC medical genetics, 5, 26-26 (2004-10-28)
Cystic fibrosis (CF) lung disease manifest by impaired chloride secretion leads to eventual respiratory failure. Candidate genes that may modify CF lung disease severity include alternative chloride channels. The objectives of this study are to identify single nucleotide polymorphisms (SNPs)
E Stogmann et al.
Neurogenetics, 7(4), 265-268 (2006-08-26)
Mutations in the chloride channel gene CLCN2 have been reported in families with generalized and focal epilepsy syndromes. To evaluate the contribution of mutations in the CLCN2 gene to the etiology of epilepsies in our population, we screened 96 patients
María Isabel Niemeyer et al.
Physiological genomics, 19(1), 74-83 (2004-07-15)
The ClC-2 Cl- channel has been postulated to play a role in the inhibitory GABA response in neurons or to participate in astrocyte-dependent extracellular electrolyte homeostasis. Three different mutations in the CLCN2 gene, encoding the voltage-dependent homodimeric ClC-2 channel, have
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