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Mouse Anti-CLCN4 Recombinant Antibody (N222-6) (CBMAB-1382-CN)


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Summary

Host Animal
Mouse
Specificity
Human
Clone
N222/6
Antibody Isotype
IgG2a, κ
Application
WB, IHC

Basic Information

Immunogen
Recombinant CLCN4.
Host Species
Mouse
Specificity
Human
Antibody Isotype
IgG2a, κ
Clonality
Monoclonal Antibody
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
PBS
Preservative
None
Concentration
1 mg/ml
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 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. The physiological role of chloride channel 4 remains unknown but may contribute to the pathogenesis of neuronal disorders. Proton-coupled chloride transporterThis protein functions as antiport system and exchanges chloride ions against protons.
Entrez Gene ID
1183
UniProt ID
P51793
Alternative Names
CLC4; ClC-4; MRX15; MRX49; ClC-4A
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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