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

Name: Mouse Anti-CLCN4 Recombinant Antibody (N222-6)
SKU: CBMAB-1382-CN
Rating: 5 (1 reviews)

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Datasheet

SDS

Request for COA

Datasheet Target References Q & As Review & reward Protocols Associated Products

Basic Information

Host Animal

Mouse

Clone

N222/6

Application

WB, IHC

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.

More Infomation

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

Kim, D., Do, H., Son, G., Kim, Y., Jeon, S., & Han, J. (2019, September). Biological roles of CLCN4 in human neuronal development. In 10th World Congress of Neuroscience (International Brain Research Organization; IBRO 2019) (pp. S467-S468). International Brain Research Organization, Federation of Asian-Oceanian Neuroscience Societies.

Palmer, E. E., Stuhlmann, T., Weinert, S., Haan, E., Van Esch, H., Holvoet, M., ... & Kalscheuer, V. M. (2018). De novo and inherited mutations in the X-linked gene CLCN4 are associated with syndromic intellectual disability and behavior and seizure disorders in males and females. Molecular psychiatry, 23(2), 222-230.

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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

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Isotype control

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

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