Rabbit anti-Human KCNJ13 Polyclonal Antibody | anti-KCNJ13 antibody

KCNJ13, NT (KCNJ13, Inward rectifier potassium channel 13, Inward rectifier K(+) channel Kir7.1, Potassium channel, inwardly rectifying subfamily J member 13) (APC)

Cat. Num. AAA6312534

• Gene Names: KCNJ13; SVD; LCA16; KIR1.4; KIR7.1
• Reactivity: Human
• Applications: Immunohistochemistry, Western Blot
• Purity: Purified by Protein A and Peptide Affinity Chromatography.
• Synonyms: KCNJ13; Polyclonal Antibody; NT (KCNJ13; Inward rectifier potassium channel 13; Inward rectifier K(+) channel Kir7.1; Potassium channel; inwardly rectifying subfamily J member 13) (APC); inwardly rectifying subfamily J member 13; anti-KCNJ13 antibody

• Host: Rabbit
• Reactivity: Human
• Clonality: Polyclonal
• Isotype: IgG
• Specificity: Recognizes human KCNJ13.
• Purity/Purification: Purified by Protein A and Peptide Affinity Chromatography.
• Form/Format: Supplied as a liquid in PBS, pH 7.2. No preservative added. Labeled with Allophycocyanin (APC).
• Sequence Length: 360

• Applicable Applications for anti-KCNJ13 antibody: Immunohistochemistry (IHC) Paraffin, Western Blot (WB), FLISA
• Application Notes: Applications are based on unconjugated antibody.
• Immunogen: KLH-conjugated synthetic peptide mapping to a fragment of residues within amino acids 67-95 in the N-terminal region of human KCNJ13, UniProt Accession #O60928.
• Conjugate: APC
• Note: Preservative Free
• Special Handling: Light sensitive
• Preparation and Storage: Store product at 4 degree C in the dark. DO NOT FREEZE! Stable at 4 degree C for 12 months after receipt as an undiluted liquid. Dilute required amount only prior to immediate use. Further dilutions can be made in assay buffer. Caution: APC conjugates are sensitive to light. For maximum recovery of product, centrifuge the original vial prior to removing the cap.

Western Blot (WB)

(Western Blot analysis in MDA-MB435 cell line lysates (35ug/lane) usingMBS6008039. This demonstrates that MBS6008039 detected the KCNJ13 protein (arrow).)

Immunohistochemistry (IHC)

(Immunohistochemistry analysis in formalin fixed and paraffin embedded human stomach tissue using MBS6008039 followed by peroxidase conjugation of the secondary antibody and DAB staining.This data demonstrates the use of MBS6008039 for immunohistochemistry.)

Product Categories/Family for anti-KCNJ13 antibody

Antibodies; Ion Channel

References

1. Zhang, W., et al. Biochem. Biophys. Res. Commun. 377(3): 981-986(2008). 2. Ji, W., et al. Nat. Genet. 40(5):592-599 (2008). 3. Hughes, B.A., et al. Am. J. Physiol., Cell Physiol. 294 (2), C423-C431 (2008). 4. Hejtmancik, J.F., et al. Am. J. Hum. Genet. 82(1):174-180 (2008). 5. Yang, D., et al. Exp. Eye Res. 86(1):81-91(2008).

NCBI and Uniprot Product Information

• NCBI GI #: 156119627
• NCBI GeneID: 3769
• NCBI Accession #: NP_002233.2; NP_001165887.1
• NCBI GenBank Nucleotide #: NP_002233.2
• UniProt Accession #: O60928
• NCBI Official Full Name: inward rectifier potassium channel 13 isoform 1
• NCBI Official Synonym Full Names: potassium inwardly rectifying channel subfamily J member 13
• NCBI Official Symbol: KCNJ13
• NCBI Official Synonym Symbols: SVD; LCA16; KIR1.4; KIR7.1
• NCBI Protein Information: inward rectifier potassium channel 13
• UniProt Protein Name: Inward rectifier potassium channel 13
• Protein Family: Inward rectifier potassium channel
• UniProt Gene Name: KCNJ13
• UniProt Entry Name: KCJ13_HUMAN

NCBI Description

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]

Uniprot Description

KCNJ13: 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. Defects in KCNJ13 are the cause of snowflake vitreoretinal degeneration (SVD). SVD is a 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. Defects in KCNJ13 are the cause of Leber congenital amaurosis type 16 (LCA16). LCA16 is 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. Belongs to the inward rectifier-type potassium channel (TC 1.A.2.1) family. KCNJ13 subfamily. 2 isoforms of the human protein are produced by alternative splicing.

Protein type: Membrane protein, multi-pass; Membrane protein, integral; Channel, potassium

Chromosomal Location of Human Ortholog: 2q37

Cellular Component: integral to plasma membrane

Molecular Function: inward rectifier potassium channel activity

Biological Process: potassium ion import; potassium ion transport

Disease: Leber Congenital Amaurosis 16; Vitreoretinal Degeneration, Snowflake Type