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

This product is antibody recognizes KCNJ13. The antibody CBLY1-052 immunoassay techniques such as: WB, IP, IF, ELISA.
See all KCNJ13 antibodies

Summary

Host Animal
Mouse
Specificity
Human
Clone
CBLY1-052
Antibody Isotype
IgG
Application
WB, IP, IF, ELISA

Basic Information

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.

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

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