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Mouse Anti-KCNA2 Recombinant Antibody (11C244) (CBMAB-K0471-LY)

This product is antibody recognizes KCNA2. The antibody 11C244 immunoassay techniques such as: WB, IHC, IF.
See all KCNA2 antibodies

Summary

Host Animal
Mouse
Specificity
Rat
Clone
11C244
Antibody Isotype
IgG2b, κ
Application
WB, IHC, IF

Basic Information

Immunogen
Recognizes rat Kv1.2
Specificity
Rat
Antibody Isotype
IgG2b, κ
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
Preservative
0.05% sodium azide
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 A Member 2
Introduction
Potassium channels represent the most complex class of voltage-gated ion channels from both functional and structural standpoints. Their diverse functions include regulating neurotransmitter release, heart rate, insulin secretion, neuronal excitability, epithelial electrolyte transport, smooth muscle contraction, and cell volume. Four sequence-related potassium channel genes - shaker, shaw, shab, and shal - have been identified in Drosophila, and each has been shown to have human homolog(s). This gene encodes a member of the potassium channel, voltage-gated, shaker-related subfamily. This member contains six membrane-spanning domains with a shaker-type repeat in the fourth segment. It belongs to the delayed rectifier class, members of which allow nerve cells to efficiently repolarize following an action potential. The coding region of this gene is intronless, and the gene is clustered with genes KCNA3 and KCNA10 on chromosome 1. [provided by RefSeq, Jul 2008]
Entrez Gene ID
UniProt ID
Function
Voltage-gated potassium channel that mediates transmembrane potassium transport in excitable membranes, primarily in the brain and the central nervous system, but also in the cardiovascular system. Prevents aberrant action potential firing and regulates neuronal output. Forms tetrameric potassium-selective channels through which potassium ions pass in accordance with their electrochemical gradient. The channel alternates between opened and closed conformations in response to the voltage difference across the membrane (PubMed:19912772, PubMed:8495559, PubMed:11211111, PubMed:23769686).
Can form functional homotetrameric channels and heterotetrameric channels that contain variable proportions of KCNA1, KCNA2, KCNA4, KCNA5, KCNA6, KCNA7, and possibly other family members as well; channel properties depend on the type of alpha subunits that are part of the channel (PubMed:8495559, PubMed:20220134).
Channel properties are modulated by cytoplasmic beta subunits that regulate the subcellular location of the alpha subunits and promote rapid inactivation of delayed rectifier potassium channels. In vivo, membranes probably contain a mixture of heteromeric potassium channel complexes, making it difficult to assign currents observed in intact tissues to any particular potassium channel family member. Homotetrameric KCNA2 forms a delayed-rectifier potassium channel that opens in response to membrane depolarization, followed by slow spontaneous channel closure (PubMed:19912772, PubMed:23769686).
In contrast, a heteromultimer formed by KCNA2 and KCNA4 shows rapid inactivation (PubMed:8495559).
Regulates neuronal excitability and plays a role as pacemaker in the regulation of neuronal action potentials (By similarity).
KCNA2-containing channels play a presynaptic role and prevent hyperexcitability and aberrant action potential firing (By similarity).
Response to toxins that are selective for KCNA2-containing potassium channels suggests that in Purkinje cells, dendritic subthreshold KCNA2-containing potassium channels prevent random spontaneous calcium spikes, suppressing dendritic hyperexcitability without hindering the generation of somatic action potentials, and thereby play an important role in motor coordination (By similarity).
Plays a role in the induction of long-term potentiation of neuron excitability in the CA3 layer of the hippocampus (By similarity).
May function as down-stream effector for G protein-coupled receptors and inhibit GABAergic inputs to basolateral amygdala neurons (By similarity).
May contribute to the regulation of neurotransmitter release, such as gamma-aminobutyric acid (GABA) (By similarity).
Contributes to the regulation of the axonal release of the neurotransmitter dopamine (By similarity).
Reduced KCNA2 expression plays a role in the perception of neuropathic pain after peripheral nerve injury, but not acute pain (By similarity).
Plays a role in the regulation of the time spent in non-rapid eye movement (NREM) sleep (By similarity).
Biological Process
Neuronal action potentialISS:UniProtKB
Optic nerve structural organizationIEA:Ensembl
Potassium ion export across plasma membraneIEA:Ensembl
Potassium ion transmembrane transportManual Assertion Based On ExperimentIMP:UniProtKB
Potassium ion transportManual Assertion Based On ExperimentTAS:ProtInc
Protein homooligomerizationIEA:InterPro
Regulation of circadian sleep/wake cycle, non-REM sleepIEA:Ensembl
Regulation of dopamine secretionISS:UniProtKB
Regulation of ion transmembrane transportIEA:UniProtKB-KW
Sensory perception of painISS:UniProtKB
Cellular Location
Cell membrane; Membrane; Cell projection, axon; Cytoplasmic vesicle; Perikaryon; Endoplasmic reticulum; Cell projection, dendrite; Cell junction; Cell junction, synapse; Cell junction, synapse, presynaptic cell membrane; Cell junction, synapse, presynapse. Homotetrameric KCNA1 is primarily located in the endoplasmic reticulum. Interaction with KCNA2 and KCNAB2 or with KCNA4 and KCNAB2 promotes expression at the cell membrane (By similarity).
Involvement in disease
Developmental and epileptic encephalopathy 32 (DEE32):
A form of epileptic encephalopathy, a heterogeneous group of severe early-onset epilepsies characterized by refractory seizures, neurodevelopmental impairment, and poor prognosis. Development is normal prior to seizure onset, after which cognitive and motor delays become apparent. DEE32 inheritance is autosomal dominant.
Topology
Cytoplasmic: 1-160
Helical: 161-182
Extracellular: 183-221
Helical: 222-243
Cytoplasmic: 244-254
Helical: 255-275
Extracellular: 276-289
Helical: 290-310
Cytoplasmic: 311-325
Helical: 326-347
Extracellular: 348-361
Helical: 362-381
Extracellular: 382-388
Helical: 389-417
Cytoplasmic: 418-499
PTM
Phosphorylated on tyrosine residues; phosphorylation increases in response to ischemia (By similarity).
Phosphorylated on tyrosine residues by activated PTK2B/PYK2 (By similarity).
Phosphorylation on tyrosine residues suppresses ion channel activity (By similarity).
Phosphorylated on tyrosine residues in response to CHRM1 activation; this abolishes interaction with CTTN. This is probably due to endocytosis of the phosphorylated channel subunits (By similarity).
Phosphorylated on serine residues in response to increased cAMP levels; phosphorylation is apparently not catalyzed by PKA (By similarity).
N-glycosylated, with complex, sialylated N-glycans.By Similarity

Hully, M., Lo Barco, T., Kaminska, A., Barcia, G., Cances, C., Mignot, C., ... & Nabbout, R. (2021). Deep phenotyping unstructured data mining in an extensive pediatric database to unravel a common KCNA2 variant in neurodevelopmental syndromes. Genetics in Medicine, 23(5), 968-971.

Timäus, C., von Gottberg, P., Hirschel, S., Lange, C., Wiltfang, J., & Hansen, N. (2021). KCNA2 autoimmunity in progressive cognitive impairment: case series and literature review. Brain Sciences, 11(1), 89.

Imbrici, P., Conte, E., Blunck, R., Stregapede, F., Liantonio, A., Tosi, M., ... & Zanni, G. (2021). A novel KCNA2 variant in a patient with non-progressive congenital ataxia and epilepsy: functional characterization and sensitivity to 4-aminopyridine. International Journal of Molecular Sciences, 22(18), 9913.

Hedrich, U. B., Lauxmann, S., Wolff, M., Synofzik, M., Bast, T., Binelli, A., ... & Lerche, H. (2021). 4-Aminopyridine is a promising treatment option for patients with gain-of-function KCNA2-encephalopathy. Science translational medicine, 13(609), eaaz4957.

Döring, J. H., Schröter, J., Jüngling, J., Biskup, S., Klotz, K. A., Bast, T., ... & Syrbe, S. (2021). Refining genotypes and phenotypes in KCNA2-related neurological disorders. International Journal of Molecular Sciences, 22(6), 2824.

Kong, C., Du, J., Bu, H., Huang, C., Xu, F., & Ren, H. (2020). LncRNA KCNA2-AS regulates spinal astrocyte activation through STAT3 to affect postherpetic neuralgia. Molecular Medicine, 26, 1-12.

Gong, P., Jiao, X., Zhang, Y., & Yang, Z. (2020). Complex mosaicism of two distinct mutations in a female patient with KCNA2-related encephalopathy: a case report. Frontiers in Genetics, 11, 911.

Sun, L., Gu, X., Pan, Z., Guo, X., Liu, J., Atianjoh, F. E., ... & Tao, Y. X. (2019). Contribution of DNMT1 to neuropathic pain genesis partially through epigenetically repressing Kcna2 in primary afferent neurons. Journal of Neuroscience, 39(33), 6595-6607.

Rogers, A., Golumbek, P., Cellini, E., Doccini, V., Guerrini, R., Wallgren‐Pettersson, C., ... & Gurnett, C. A. (2018). De novo KCNA1 variants in the PVP motif cause infantile epileptic encephalopathy and cognitive impairment similar to recurrent KCNA2 variants. American Journal of Medical Genetics Part A, 176(8), 1748-1752.

Mo, K., Wu, S., Gu, X., Xiong, M., Cai, W., Atianjoh, F. E., ... & Tao, Y. X. (2018). MBD1 contributes to the genesis of acute pain and neuropathic pain by epigenetic silencing of Oprm1 and Kcna2 genes in primary sensory neurons. Journal of Neuroscience, 38(46), 9883-9899.

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

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