HPA018305
Anti-KCNQ4 antibody produced in rabbit
Prestige Antibodies® Powered by Atlas Antibodies, affinity isolated antibody, buffered aqueous glycerol solution
Synonym(s):
Anti-KQT-like 4, Anti-Potassium channel subunit alpha KvLQT4, Anti-Potassium voltage-gated channel subfamily KQT member 4, Anti-Voltage-gated potassium channel subunit Kv74
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All Photos(3)
About This Item
UNSPSC Code:
12352203
Human Protein Atlas Number:
biological source
rabbit
conjugate
unconjugated
antibody form
affinity isolated antibody
antibody product type
primary antibodies
clone
polyclonal
product line
Prestige Antibodies® Powered by Atlas Antibodies
form
buffered aqueous glycerol solution
species reactivity
human
technique(s)
immunohistochemistry (formalin-fixed, paraffin-embedded sections): suitable
western blot: suitable
immunogen sequence
REKGDKGPSDAEVVDEISMMGRVVKVEKQVQSIEHKLDLLLGFYSRCLRSGTSASLGAVQVPLFDPDITSDYHSPVDHEDISVSAQTLSISRSV
UniProt accession no.
shipped in
wet ice
storage temp.
−20°C
target post-translational modification
unmodified
Gene Information
human ... KCNQ4(9132)
General description
The gene potassium voltage-gated channel subfamily KQT member-4 (KCNQ4) is mapped to human chromosome 1p34. It belongs to KCNQ family of K+ channels and forms heteromeric complexes with KCNQ3. KCNQ4 transcripts are detected in heart, brain, and skeletal muscle. KCNQ4 is also strongly expressed in the inner ear and the central auditory pathway. In the inner ear, KCNQ4 is particularly present in the basal membrane of sensory outer hair cells. KCNQ4 has six transmembrane domains, a pore forming region and two intracellular termini. The protein is localized in the plasma membrane.
Immunogen
Potassium voltage-gated channel subfamily KQT member 4 recombinant protein epitope signature tag (PrEST)
Application
All Prestige Antibodies Powered by Atlas Antibodies are developed and validated by the Human Protein Atlas (HPA) project and as a result, are supported by the most extensive characterization in the industry.
The Human Protein Atlas project can be subdivided into three efforts: Human Tissue Atlas, Cancer Atlas, and Human Cell Atlas. The antibodies that have been generated in support of the Tissue and Cancer Atlas projects have been tested by immunohistochemistry against hundreds of normal and disease tissues and through the recent efforts of the Human Cell Atlas project, many have been characterized by immunofluorescence to map the human proteome not only at the tissue level but now at the subcellular level. These images and the collection of this vast data set can be viewed on the Human Protein Atlas (HPA) site by clicking on the Image Gallery link. We also provide Prestige Antibodies® protocols and other useful information.
The Human Protein Atlas project can be subdivided into three efforts: Human Tissue Atlas, Cancer Atlas, and Human Cell Atlas. The antibodies that have been generated in support of the Tissue and Cancer Atlas projects have been tested by immunohistochemistry against hundreds of normal and disease tissues and through the recent efforts of the Human Cell Atlas project, many have been characterized by immunofluorescence to map the human proteome not only at the tissue level but now at the subcellular level. These images and the collection of this vast data set can be viewed on the Human Protein Atlas (HPA) site by clicking on the Image Gallery link. We also provide Prestige Antibodies® protocols and other useful information.
Biochem/physiol Actions
HSP40 (Heat shock protein 40)-HSP70-HOP (HSP70-HSP90 organizing protein)-HSP90 chaperone pathway is responsible for maintaining potassium voltage-gated channel KQT member-4 (KCNQ4) homeostasis in the cell. Mutations in KCNQ4 are associated with autosomal dominant hearing loss. In addition, KCNQ4 plays protective role in the pulmonary circulation and prevents hypoxia-induced pulmonary circulation. KCNQ4 is important for myogenesis. Cannabinoid receptor of type-1 represses myoblast differentiation by reducing KCNQ4 levels.
Features and Benefits
Prestige Antibodies® are highly characterized and extensively validated antibodies with the added benefit of all available characterization data for each target being accessible via the Human Protein Atlas portal linked just below the product name at the top of this page. The uniqueness and low cross-reactivity of the Prestige Antibodies® to other proteins are due to a thorough selection of antigen regions, affinity purification, and stringent selection. Prestige antigen controls are available for every corresponding Prestige Antibody and can be found in the linkage section.
Every Prestige Antibody is tested in the following ways:
Every Prestige Antibody is tested in the following ways:
- IHC tissue array of 44 normal human tissues and 20 of the most common cancer type tissues.
- Protein array of 364 human recombinant protein fragments.
Linkage
Corresponding Antigen APREST73231
Physical form
Solution in phosphate-buffered saline, pH 7.2, containing 40% glycerol and 0.02% sodium azide
Legal Information
Prestige Antibodies is a registered trademark of Merck KGaA, Darmstadt, Germany
Disclaimer
Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.
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Storage Class
10 - Combustible liquids
wgk_germany
WGK 1
flash_point_f
Not applicable
flash_point_c
Not applicable
ppe
Eyeshields, Gloves, multi-purpose combination respirator cartridge (US)
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Impaired surface expression and conductance of the KCNQ4 channel lead to sensorineural hearing loss.
Yanhong Gao et al.
Journal of cellular and molecular medicine, 17(7), 889-900 (2013-06-12)
KCNQ4, a voltage-gated potassium channel, plays an important role in maintaining cochlear ion homoeostasis and regulating hair cell membrane potential, both essential for normal auditory function. Mutations in the KCNQ4 gene lead to DFNA2, a subtype of autosomal dominant non-syndromic
Yanhong Gao et al.
PloS one, 8(2), e57282-e57282 (2013-02-23)
Loss-of-function mutations in the KCNQ4 channel cause DFNA2, a subtype of autosomal dominant non-syndromic deafness that is characterized by progressive sensorineural hearing loss. Previous studies have demonstrated that the majority of the pathogenic KCNQ4 mutations lead to trafficking deficiency and
Fabio A Iannotti et al.
Proceedings of the National Academy of Sciences of the United States of America, 111(24), E2472-E2481 (2014-06-14)
Little is known of the involvement of endocannabinoids and cannabinoid receptors in skeletal muscle cell differentiation. We report that, due to changes in the expression of genes involved in its metabolism, the levels of the endocannabinoid 2-arachidonoylglycerol (2-AG) are decreased
P J Coucke et al.
Human molecular genetics, 8(7), 1321-1328 (1999-06-17)
We have previously found linkage to chromosome 1p34 in five large families with autosomal dominant non-syndromic hearing impairment (DFNA2). In all five families, the connexin31 gene ( GJB3 ), located at 1p34 and responsible for non-syndromic autosomal dominant hearing loss
C Kubisch et al.
Cell, 96(3), 437-446 (1999-02-20)
Potassium channels regulate electrical signaling and the ionic composition of biological fluids. Mutations in the three known genes of the KCNQ branch of the K+ channel gene family underlie inherited cardiac arrhythmias (in some cases associated with deafness) and neonatal
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