CLCN2 Antibody

中文名稱：

CLCN2兔多克隆抗體
貨號：

CSB-PA005481LA01HU
規格：

￥440
圖片：
Immunofluorescent analysis of HepG2 cells using CSB-PA005481LA01HU at dilution of 1:100 and Alexa Fluor 488-congugated AffiniPure Goat Anti-Rabbit IgG(H+L)
其他：

產品詳情
產品評價
相關產品
靶點詳情

產品詳情

產品名稱：

Rabbit anti-Homo sapiens (Human) CLCN2 Polyclonal antibody
Uniprot No.：

P51788
基因名：

CLCN2
別名：

Chloride Channel 2 antibody; Chloride channel protein 2 antibody; Chloride channel; voltage sensitive 2 antibody; CIC 2 antibody; CIC2 antibody; ClC-2 antibody; CLC2 antibody; Clcn2 antibody; CLCN2_HUMAN antibody; ECA2 antibody; ECA3 antibody; EG13 antibody; EGI11 antibody; EGMA antibody; EJM6 antibody; EJM8 antibody; PKA-activated chloride channel antibody
宿主：

Rabbit
反應種屬：

Human
免疫原：

Recombinant Human Chloride channel protein 2 protein (549-770AA)
免疫原種屬：

Homo sapiens (Human)

標記方式：

Non-conjugated

本頁面中的產品，CLCN2 Antibody (CSB-PA005481LA01HU)，的標記方式是Non-conjugated。對于CLCN2 Antibody，我們還提供其他標記。見下表：

標記方式	貨號	產品名稱	應用
HRP	CSB-PA005481LB01HU	CLCN2 Antibody, HRP conjugated	ELISA
FITC	CSB-PA005481LC01HU	CLCN2 Antibody, FITC conjugated
Biotin	CSB-PA005481LD01HU	CLCN2 Antibody, Biotin conjugated	ELISA

克隆類型：

Polyclonal
抗體亞型：

IgG
純化方式：

>95%, Protein G purified
濃度：

It differs from different batches. Please contact us to confirm it.
保存緩沖液：

Preservative: 0.03% Proclin 300
Constituents: 50% Glycerol, 0.01M PBS, PH 7.4
產品提供形式：

Liquid
應用范圍：

ELISA, IF

推薦稀釋比：

Application	Recommended Dilution
IF	1:50-1:200

Protocols：

ELISA Protocol
Immunofluorescence (IF) Protocol
儲存條件：

Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
貨期：

Basically, we can dispatch the products out in 1-3 working days after receiving your orders. Delivery time maybe differs from different purchasing way or location, please kindly consult your local distributors for specific delivery time.
用途：

For Research Use Only. Not for use in diagnostic or therapeutic procedures.

產品評價

靶點詳情

功能：

Voltage-gated chloride channel. Chloride channels have several functions including the regulation of cell volume, membrane potential stabilization, signal transduction and transepithelial transport. Involved in the regulation of aldosterone production. The opening of CLCN2 channels at hyperpolarized membrane potentials in the glomerulosa causes cell membrane depolarization, activation of voltage-gated Ca2+ channels and increased expression of aldosterone synthase, the rate-limiting enzyme for aldosterone biosynthesis.
基因功能參考文獻：
1. The inhibitory effects of ClC-2 knockout on HBVSMC proliferation and motility were associated with inactivation of the Wnt/beta-catenin signaling pathway, as evidenced by inhibition of beta-catenin phosphorylation and nuclear translocation, and decrease of GSK-3beta phosphorylation and survivin and cyclin D1 expression. PMID: 29988306
2. Leukoencephalopathy-causing CLCN2 mutations impair chloride channel gating and trafficking. PMID: 28905383
3. ClC-2 mRNA level was significantly increased in patients with non-alcoholic steatohepatitis, which positively correlated with the plasma levels of alanine transaminase (ALT), aspartate transaminase (AST) and insulin. PMID: 29550812
4. The findings show that RGET691 of human ClC-2 (possible binding site) plays an important functional role in fatty acid activation of human ClC-2. PMID: 28424169
5. Hyperpolarization activates CLC-2 mainly by driving intracellular anions into the channel pores. PMID: 26666914
6. The extracellular domain of GlialCAM is necessary for cell junction targeting and for mediating interactions with itself or with MLC1 and ClC-2. PMID: 26033718
7. both ubiquitous (AP-1A) and epithelium-specific (AP-1B) forms of the tetrameric clathrin adaptor AP-1 are capable of carrying out basolateral sorting of ClC-2. PMID: 25739457
8. SPAK and OSR1 are powerful negative regulators of the cell volume regulatory Cl- channel ClC-2 PMID: 25323061
9. Correlation between CLC-2 gene expression and the cytoskeleton in human trabecular meshwork cells. PMID: 23934342
10. Our observations substantiate the concept that ClC-2 is involved in brain ion and water homoeostasis PMID: 23707145
11. JAK2 down-regulates ClC-2 activity and thus counteracts Cl(-) exit, an effect which may impact on cell volume regulation PMID: 22613974
12. This study demonistrated that the first auxiliary subunit of ClC-2 and suggests that ClC-2 may play a role in the pathology of MLC disease. PMID: 22405205
13. ClC-2 plays an important role in the modulation of tight junctions by influencing caveolar trafficking of tight junction protein occludin. PMID: 21956164
14. Sequence analysis of CLCN2 at genomic DNA and cDNA levels in 18 megalencephalic leukoencephalopathy MLC1 mutations revealed some nucleotide changes, but they were predicted to be nonpathogenic. PMID: 20187760
15. found significant expression of ClC-2 at the apex of ciliated cells in both rat and human airways PMID: 11880269
16. a functional interaction between the ClC-2 chloride channel and the retrograde motor dynein complex. PMID: 12601004
17. ClC-2 and ClC-3 channels are specifically upregulated in glioma membranes and endow glioma cells with an enhanced ability to transport Chloride PMID: 12843258
18. activation of hClC-2 is differentially regulated by PKA at two sites PMID: 15010473
19. Functional evaluation of mutated channels associated with idiopathic generalized epilepsies PMID: 15252188
20. activation of host ClC-2 channels participates in the altered permeability of Plasmodium-infected erythrocytes but is not required for intraerythrocytic parasite survival PMID: 15272009
21. These results indicate that ClC-2 may not be a Cl- -transporting protein for gastric acid secretion in parietal cells. PMID: 15388342
22. These data suggest that interferon-gamma activates ClC-2 channel activity in lung epithelial cells via mRNA stabilization. PMID: 15464978
23. The authors conclude that CLCN2 mutations may be a rare cause of familial epilepsy. Further studies are needed to test if polymorphisms in this gene are associated with epilepsy. PMID: 15505175
24. CLC-2 is not a key modifier gene of Cystic fibrosis lung disease phenotype. PMID: 15507145
25. CLCN2 is related to idiopathic generalized epilepsy. PMID: 15508929
26. association of Hsp90 with ClC-2 results in greater channel activity due to increased cell surface channel expression, facilitation of channel opening, and enhanced channel sensitivity to intracellular [Cl-] PMID: 16049054
27. This suggests that slow and fast gating in ClC-2 are coupled, perhaps with slow gating contributing to the operation of the pore E207 as a protopore gate. PMID: 16469788
28. mutations in the CLCN2 gene are only a rare cause of idiopathic generalized epilepsy PMID: 16932951
29. CLCN2 may be a susceptibility locus in a subset of cases of childhood absence epilepsy PMID: 17580110
30. Africans' gene pool comprises CLCN2 gene variants in the N-terminus, the C-terminus or the pore domain that affect surface expression and voltage- or cell-swelling-stimulated channel gating PMID: 17762171
31. CLC-2 is upregulated in ethmoid mucosa and may affect the development of chronic rhinosinusitis without nasal polyps. PMID: 17882904
32. propose that the function of the ClC-2 carboxy-terminus is to slow down the time course of channel activation in order to stabilize neuronal excitability PMID: 18801843
33. Finding predicts a loss of function that may contribute to intracellular chloride accumulation or neuronal hyperexcitability. PMID: 19191339
34. PIKfyve is a potent stimulator of ClC-2-activity and contributes to SGK1-dependent regulation of ClC-2. PMID: 19232516
35. GaTx2 specifically inhibits ClC-2 channels, showing no inhibitory effect on a battery of other major classes of chloride channels and voltage-gated potassium channels. GaTx2 is the first peptide toxin inhibitor of any ClC protein. PMID: 19574231
36. CLCN2 variants in indiopathic generalized epilepsy are reported. PMID: 19710712
37. ClC-2 requires residing in beta-cyclodextrin sensitive plasma membrane clusters with other molecules in order to remain active. Regulation of ClC-2 trafficking to (endocytic pathway) and within the membrane could be a means of modulating its activity. PMID: 19711355
38. two novel CLCN2 missense mutations, p.Arg235Gln and p.Arg577Gln, accelerating chloride channel deactivation are associated with idiopathic generalized epilepsy. PMID: 19191339
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相關疾病：

Epilepsy, idiopathic generalized 11 (EIG11); Juvenile absence epilepsy 2 (JAE2); Juvenile myoclonic epilepsy 8 (EJM8); Leukoencephalopathy with ataxia (LKPAT)
亞細胞定位：

Cell membrane; Multi-pass membrane protein.
蛋白家族：

Chloride channel (TC 2.A.49) family, ClC-2/CLCN2 subfamily
組織特異性：

Ubiquitously expressed. Moderately expressed in aortic and coronary vascular smooth muscle cells and expressed at a low level in aortic endothelial cells. Expressed in the adrenal gland, predominantly in the zona glomerulosa.
數據庫鏈接：

HGNC: 2020

OMIM: 600570

KEGG: hsa:1181

STRING: 9606.ENSP00000265593

UniGene: Hs.436847

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