Rabbit Anti-CLCN2 Recombinant Antibody (EPR6492(2)) (CBMAB-1379-CN)

This product is a rabbit antibody that recognizes CLCN2 of human. The antibody EPR6492(2) can be used for immunoassay techniques such as: IHC-P, WB.
See all CLCN2 antibodies

Datasheet

Summary

Host Animal

Rabbit

Specificity

Human, Mouse, Rat

Clone

EPR6492(2)

Antibody Isotype

IgG

Application

IHC-P, WB

Basic Information

Immunogen

Synthetic peptide corresponding to residues in Human CLCN2.

Specificity

Human, Mouse, Rat

Antibody Isotype

IgG

Clonality

Monoclonal

Application Notes

The COA includes recommended starting dilutions, optimal dilutions should be determined by the end user.

Formulations & Storage [For reference only, actual COA shall prevail!]

Format

Liquid

Buffer

50% Glycerol, 0.05% BSA

Preservative

0.01% Sodium azide

Storage

Store at +4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freeze/thaw cycles.

Target

Full Name

chloride channel 2

Introduction

This gene encodes a voltage-gated chloride channel. The encoded protein is a transmembrane protein that maintains chloride ion homeostasis in various cells. Four transcript variants encoding different isoforms have been found for this gene. Voltage-gated chloride channel. Chloride channels have several functions including the regulation of cell volume; membrane potential stabilization, signal transduction and transepithelial transport.

Entrez Gene ID

Human	1181
Mouse	12724
Rat	29232

UniProt ID

Human	P51788
Mouse	Q9R0A1
Rat	P35525

Alternative Names

CLC2; ECA2; ECA3; EGI3; EGMA; EJM6; EJM8; CIC-2; EGI11; LKPAT; clC-2

Function

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 (PubMed:29403011, PubMed:29403012).

Biological Process

Cell differentiation involved in salivary gland development Source: Ensembl
Chloride transport Source: GO_Central
Ion transmembrane transport Source: Reactome
Regulation of aldosterone biosynthetic process Source: UniProtKB
Regulation of ion transmembrane transport Source: UniProtKB-KW
Retina development in camera-type eye Source: Ensembl

Cellular Location

Cell membrane

Involvement in disease

Epilepsy, idiopathic generalized 11 (EIG11):
A disorder characterized by recurring generalized seizures in the absence of detectable brain lesions and/or metabolic abnormalities. Generalized seizures arise diffusely and simultaneously from both hemispheres of the brain.
Juvenile absence epilepsy 2 (JAE2):
A subtype of idiopathic generalized epilepsy characterized by onset occurring around puberty, absence seizures, generalized tonic-clonic seizures (GTCS), GTCS on awakening, and myoclonic seizures.
Juvenile myoclonic epilepsy 8 (EJM8):
A subtype of idiopathic generalized epilepsy. Patients have afebrile seizures only, with onset in adolescence (rather than in childhood) and myoclonic jerks which usually occur after awakening and are triggered by sleep deprivation and fatigue.
Leukoencephalopathy with ataxia (LKPAT):
An autosomal recessive neurologic disorder with a characteristic pattern of white matter abnormalities on brain MRI. Affected individuals have prominent signal abnormalities and decreased apparent diffusion coefficient values in the posterior limbs of the internal capsules, middle cerebral peduncles, pyramidal tracts in the pons, and middle cerebellar peduncles, suggesting myelin microvacuolation. Clinical features include ataxia and unstable gait. More variable abnormalities may include visual field defects, headaches, and learning disabilities.
Hyperaldosteronism, familial, 2 (HALD2):
An autosomal dominant disorder characterized by elevated plasma aldosterone level and hypertension of varying severity even within members of the same family. Hypokalemia is observed in some patients. In HALD2, hypertension does not improve with glucocorticoid treatment.

Topology

Cytoplasmic: 2-87
Helical: 88-121
Helical: 130-155
Helical: 164-171
Helical: 180-198
Helical: 205-223
Helical: 239-251
Helical: 255-263
Helical: 275-295
Helical: 321-349
Helical: 358-377
Helical: 429-449
Helical: 457-480
Helical: 497-511
Loop between two helices: 512-513
Helical: 514-525
Loop between two helices: 526-530
Helical: 531-548
Cytoplasmic: 549-898

PTM

Phosphorylated. Activated by dephosphorylation.

References

Tang, J., Shen, X., Yang, Y., Yang, H., Qi, A., Yang, S., ... & Chen, H. (2022). Two Different Copy Number Variations of the CLCN2 Gene in Chinese Cattle and Their Association with Growth Traits. Animals, 12(1), 41.

Rege, J., Nanba, K., Blinder, A. R., Plaska, S., Udager, A. M., Vats, P., ... & Else, T. (2020). Identification of somatic mutations in CLCN2 in aldosterone-producing adenomas. Journal of the Endocrine Society, 4(10), bvaa123.

Hansen, T. H., Yan, Y., Ahlberg, G., Vad, O. B., Refsgaard, L., Dos Santos, J. L., ... & Schmitt, N. (2020). A novel loss-of-function variant in the chloride ion channel gene Clcn2 associates with atrial fibrillation. Scientific reports, 10(1), 1-10.

Dutta, R. K., Arnesen, T., Heie, A., Walz, M., Alesina, P., Söderkvist, P., & Gimm, O. (2019). A somatic mutation in CLCN2 identified in a sporadic aldosterone-producing adenoma. European journal of endocrinology, 181(5), K37-K41.

Scholl, U. I., Stölting, G., Schewe, J., Thiel, A., Tan, H., Nelson-Williams, C., ... & Lifton, R. P. (2018). CLCN2 chloride channel mutations in familial hyperaldosteronism type II. Nature genetics, 50(3), 349-354.

Fernandes-Rosa, F. L., Daniil, G., Orozco, I. J., Göppner, C., El Zein, R., Jain, V., ... & Zennaro, M. C. (2018). A gain-of-function mutation in the CLCN2 chloride channel gene causes primary aldosteronism. Nature genetics, 50(3), 355-361.

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Protocols

Please try the standard protocols which include: protocols, troubleshooting and guide.

Enzyme-linked Immunosorbent Assay (ELISA)
Flow Cytometry
Immunofluorescence (IF)
Immunohistochemistry (IHC)
Immunoprecipitation (IP)
Western Blot (WB)
Enzyme Linked Immunospot (ELISpot)
Proteogenomic
Other Protocols

Associated Products

Related Products

Mouse Anti-CLCN2 Recombinant Antibody (3E1)

Isotype control

For research use only. Not intended for any clinical use.

Custom Antibody Labeling

We also offer labeled antibodies developed using our catalog antibody products and nonfluorescent conjugates (HRP, AP, Biotin, etc.) or fluorescent conjugates (Alexa Fluor, FITC, TRITC, Rhodamine, Texas Red, R-PE, APC, Qdot Probes, Pacific Dyes, etc.).

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