Mouse Anti-KCNJ13 Recombinant Antibody (CBLY1-052) (CBMAB-K0564-LY)

Name: Mouse Anti-KCNJ13 Recombinant Antibody (CBLY1-052)
SKU: CBMAB-K0564-LY
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

CBLY1-052

Application

WB, IP, IF, ELISA

Specificity

Human

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

Purity

> 95% Purity determined by SDS-PAGE.

Storage

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

More Infomation

Target

Full Name

Potassium Voltage-Gated Channel Subfamily J Member 13

Introduction

This gene encodes a member of the inwardly rectifying potassium channel family of proteins. Members of this family form ion channel pores that allow potassium ions to pass into a cell. The encoded protein belongs to a subfamily of low signal channel conductance proteins that have a low dependence on potassium concentration. Mutations in this gene are associated with snowflake vitreoretinal degeneration. Alternate splicing results in multiple transcript variants.[provided by RefSeq, Feb 2010]

Entrez Gene ID

3769

UniProt ID

O60928

Alternative Names

Potassium Voltage-Gated Channel Subfamily J Member 13; Potassium Inwardly-Rectifying Channel; Subfamily J; Member 13; Inward Rectifier K(+) Channel Kir7.1; Potassium Channel; Inwardly Rectifying Subfamily J; Member 13; Potassium Channel; Inwardly Rectifying Subfamily J Member 13; Inward Rectifier Potassium Channel 13;

Function

Inward rectifier potassium channels are characterized by a greater tendency to allow potassium to flow into the cell rather than out of it. Their voltage dependence is regulated by the concentration of extracellular potassium; as external potassium is raised, the voltage range of the channel opening shifts to more positive voltages. The inward rectification is mainly due to the blockage of outward current by internal magnesium. KCNJ13 has a very low single channel conductance, low sensitivity to block by external barium and cesium, and no dependence of its inward rectification properties on the internal blocking particle magnesium.

Biological Process

Potassium ion import across plasma membraneManual Assertion Based On ExperimentIBA:GO_Central
Potassium ion transport1 PublicationNAS:UniProtKB
Regulation of ion transmembrane transportManual Assertion Based On ExperimentIBA:GO_Central

Cellular Location

Membrane

Involvement in disease

Snowflake vitreoretinal degeneration (SVD):
Developmental and progressive hereditary eye disorder that affects multiple tissues within the eye. Diagnostic features of SVD include fibrillar degeneration of the vitreous humor, early-onset cataract, minute crystalline deposits in the neurosensory retina, and retinal detachment.
Leber congenital amaurosis 16 (LCA16):
A severe dystrophy of the retina, typically becoming evident in the first years of life. Visual function is usually poor and often accompanied by nystagmus, sluggish or near-absent pupillary responses, photophobia, high hyperopia and keratoconus.

Topology

Cytoplasmic: 1-53
Helical: 54-78
Extracellular: 79-105
Helical: 106-117
Pore-forming: 118-124
Extracellular: 125-133
Helical: 134-155
Cytoplasmic: 156-360

PTM

Phosphorylation at Ser-201 by PKC strongly inhibits ionic currents, while phosphorylation at Ser-287 by PKA increases them.

Kabra, M., Shahi, P. K., Wang, Y., Sinha, D., Spillane, A., Newby, G. A., ... & Pattnaik, B. R. (2023). Nonviral base editing of KCNJ13 mutation preserves vision in a model of inherited retinal channelopathy. The Journal of Clinical Investigation, 133(19).

Jiao, X., Ma, Z., Lei, J., Liu, P., Cai, X., Shahi, P. K., ... & Hejtmancik, J. F. (2022). Retinal development and pathophysiology in Kcnj13 knockout mice. Frontiers in Cell and Developmental Biology, 9, 810020.

Schroeder, M., Peter, V. G., Gränse, L., Andréasson, S., Rivolta, C., & Kjellström, U. (2022). A novel phenotype associated with the R162W variant in the KCNJ13 gene. Ophthalmic Genetics, 43(4), 500-507.

Kanzaki, Y., Fujita, H., Sato, K., Hosokawa, M., Matsumae, H., Morizane, Y., & Ohuchi, H. (2022). Protrusion of KCNJ13 Gene Knockout Retinal Pigment Epithelium Due to Oxidative Stress–Induced Cell Death. Investigative Ophthalmology & Visual Science, 63(12), 29-29.

Kanzaki, Y., Fujita, H., Sato, K., Hosokawa, M., Matsumae, H., Shiraga, F., ... & Ohuchi, H. (2020). KCNJ13 gene deletion impairs cell alignment and phagocytosis in retinal pigment epithelium derived from human-induced pluripotent stem cells. Investigative Ophthalmology & Visual Science, 61(5), 38-38.

Podobnik, M., Frohnhöfer, H. G., Dooley, C. M., Eskova, A., Nüsslein-Volhard, C., & Irion, U. (2020). Evolution of the potassium channel gene Kcnj13 underlies colour pattern diversification in Danio fish. Nature communications, 11(1), 6230.

Toms, M., Burgoyne, T., Tracey-White, D., Richardson, R., Dubis, A. M., Webster, A. R., ... & Moosajee, M. (2019). Phagosomal and mitochondrial alterations in RPE may contribute to KCNJ13 retinopathy. Scientific reports, 9(1), 3793.

Toms, M., Dubis, A. M., Lim, W. S., Webster, A. R., Gorin, M. B., & Moosajee, M. (2019). Missense variants in the conserved transmembrane M2 protein domain of KCNJ13 associated with retinovascular changes in humans and zebrafish. Experimental Eye Research, 189, 107852.

Roman, D., Zhong, H., Yaklichkin, S., Chen, R., & Mardon, G. (2018). Conditional loss of Kcnj13 in the retinal pigment epithelium causes photoreceptor degeneration. Experimental eye research, 176, 219-226.

Yin, W., Kim, H. T., Wang, S., Gunawan, F., Wang, L., Kishimoto, K., ... & Stainier, D. Y. (2018). The potassium channel KCNJ13 is essential for smooth muscle cytoskeletal organization during mouse tracheal tubulogenesis. Nature communications, 9(1), 2815.

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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
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Isotype control

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

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