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Rabbit Anti-CLCN4 Recombinant Antibody (EG716) (CBMAB-EN870-LY)

The product is antibody recognizes CLCN4. The antibody EG716 immunoassay techniques such as: WB: 1:500~1:1000 IF: 1:100~1:500 ELISA: 1:60000.
See all CLCN4 antibodies

Summary

Host Animal
Rabbit
Specificity
Human, Mouse, Rat
Clone
EG716
Antibody Isotype
IgG
Application
WB: 1:500~1:1000 IF: 1:100~1:500 ELISA: 1:60000

Basic Information

Immunogen
The antibody was produced against synthesized peptide derived from internal of human CLCN4.
Specificity
Human, Mouse, Rat
Antibody Isotype
IgG
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
Storage
Store at +4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freezethaw cycles.

Target

Full Name
Chloride Voltage-Gated Channel 4
Introduction
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. Chloride channel 4 has an evolutionary conserved CpG island and is conserved in both mouse and hamster. This gene is mapped in close proximity to APXL (Apical protein Xenopus laevis-like) and OA1 (Ocular albinism type I), which are both located on the human X chromosome at band p22.3. The physiological role of chloride channel 4 remains unknown but may contribute to the pathogenesis of neuronal disorders. Alternate splicing results in two transcript variants that encode different proteins. [provided by RefSeq, Mar 2012]
Entrez Gene ID
Human1183
Mouse12727
Rat60586
UniProt ID
HumanP51793
MouseQ61418
RatP51794
Alternative Names
Chloride Voltage-Gated Channel 4; Chloride Channel, Voltage-Sensitive 4; Chloride Channel Protein 4; Chloride Transporter ClC-4; Chloride Channel 4; ClC-4;
Function
Strongly outwardly rectifying, electrogenic H+/Cl-exchanger which mediates the exchange of chloride ions against protons (PubMed:18063579, PubMed:28972156, PubMed:23647072, PubMed:27550844, PubMed:25644381).

The CLC channel family contains both chloride channels and proton-coupled anion transporters that exchange chloride or another anion for protons (PubMed:29845874).

The presence of conserved gating glutamate residues is typical for family members that function as antiporters (PubMed:29845874).
Biological Process
Chloride transport Source: MGI
Ion transmembrane transport Source: Reactome
Cellular Location
Endoplasmic reticulum membrane; Early endosome membrane; Late endosome membrane; Recycling endosome membrane; Lysosome membrane. Localizes to late endosome membrane, lysosome membrane and recycling endosome membrane in the presence of CLCN3.
Involvement in disease
Raynaud-Claes syndrome (MRXSRC):
An X-linked syndrome characterized by borderline to severe intellectual disability and impaired language development. Additional features include behavioral problems, psychiatric disorders, seizures, progressive ataxia, brain abnormalities, and facial dysmorphisms.
Topology
Cytoplasmic: 1-67
Helical: 68-105
Helical: 151-174
Helical: 183-190
Helical: 200-218
Helical: 224-243
Helical: 255-267
Helical: 271-279
Helical: 291-309
Helical: 333-358
Helical: 365-385
Helical: 442-462
Helical: 467-486
Helical: 514-528
Helical: 532-543
Loop between two helices: 544-547
Helical: 548-566
Cytoplasmic: 567-760
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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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