mProX™ Human KCNA2 Stable Cell Line

Product Information

Target Protein

KCNA2

Target Family

Kv1

Target Protein Species

Human

Host Cell Type

CHO-K1; HEK293

Target Classification

Ion Channel Cell Lines

Target Research Area

CNS Research; Digestive and Renal Research

Related Diseases

Developmental And Epileptic Encephalopathy; Agenesis Of Corpus Callosum, Cardiac, Ocular; Genital Syndrome

Gene ID

3737

UniProt ID

P16389

Product Properties

Biosafety Level

Level 1

Activity

Yes

Quantity

10⁶ cells per vial

Applications

An alpha subunit of the voltage-gated potassium channel Kv1.2 is encoded by KCNA2. However, milder phenotypes with autosomal dominant inheritance have been documented, and both loss and gain of function mutations may have a phenotype involving epilepsy. It primarily exists as a de novo mutation. Variable phenotypes of KCNA2, such as progressive myoclonic epilepsy or epileptic encephalopathy, have been identified. In case series, it has been demonstrated that treatment with 4-aminopyridine at doses of 0.15-2.6 mg/kg/day results in seizure freedom in a number of patients. Even though these patients weren't seizure-free, there was a noticeable decrease in generalized tonic-clonic seizures. Patients who had only generalized tonic-clonic seizures prior to the administration of 4-aminopyridine were an exception to the therapeutic response; these patients did not benefit from a seizure response to the medication. The customized KCNA2 stable cell line can be used in antibody discovery and development, potential drug candidate screening and signaling pathway researches.

Protocols

Please visit our protocols page.

Customer Reviews

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I purchased the Human KCNA2 Stable Cell Line hoping it would meet my project requirements. Dec 26 2022

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The Human KCNA2 Stable Cell Line meets my requirement. Mar 05 2022

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FAQ

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Published Data

Fig.1 Identification and expression of Kcna2 antisense RNA in naive dorsal root ganglion.

By employing reverse transcription (RT)-PCR with strand-specific primers, native Kcna2 antisense (AS) transcripts were found in the DRGs of rat (Sprague-Dawley), mouse (C57/BL6), monkey (Macaca fascicularis), and human.

Ref: Zhao, Xiuli, et al. "A long noncoding RNA contributes to neuropathic pain by silencing Kcna2 in primary afferent neurons." Nature neuroscience 16.8 (2013): 1024-1031.

Pubmed: 23792947

DOI: 10.1038/nn.3438

Research Highlights

De novo mutations in the KCNA2 gene have been reported to induce a novel molecular entity within the epileptic encephalopathies. These mutations can result in either a gain-of-function or a dominant-negative loss-of-function of the voltage-gated K+ channel Kv1.2.
Masnada, Silvia, et al. "Clinical spectrum and genotype-phenotype associations of KCNA2-related encephalopathies." Brain 140.9 (2017): 2337-2354.
Pubmed: 29050392 DOI: 10.1093/brain/awx184

To investigate the phenotypic range of KCNA2 mutations and to determine the genetic basis of a family that segregates infantile seizures, diverse epilepsies, and episodic ataxia.
Corbett, Mark A., et al. "Dominant KCNA2 mutation causes episodic ataxia and pharmacoresponsive epilepsy." Neurology 87.19 (2016): 1975-1984.
Pubmed: 27733563 DOI: 10.1212/WNL.0000000000003309