KCNQ2 antibody - middle region (ARP35459_T100)
- Known as:
- KCNQ2 (anti-) - middle region (ARP35459_T100)
- Catalog number:
- arp35459_t100
- Product Quantity:
- USD
- Category:
- -
- Supplier:
- Aviva Systems Biology
- Gene target:
- KCNQ2 antibody - middle region (ARP35459_T100)
Related genes to: KCNQ2 antibody - middle region (ARP35459_T100)
- Gene:
- KCNQ2 NIH gene
- Name:
- potassium voltage-gated channel subfamily Q member 2
- Previous symbol:
- EBN, EBN1
- Synonyms:
- Kv7.2, ENB1, BFNC, KCNA11, HNSPC
- Chromosome:
- 20q13.33
- Locus Type:
- gene with protein product
- Date approved:
- 1998-01-12
- Date modifiied:
- 2016-02-04
Related products to: KCNQ2 antibody - middle region (ARP35459_T100)
Related articles to: KCNQ2 antibody - middle region (ARP35459_T100)
- The non-inactivating potassium channels Kv7.2 and Kv7.3 contribute to the neuronal resting membrane potential and regulate neuronal excitability. Previous studies have indicated a transcriptional downregulation of these channels in temporal lobe epilepsy, both in patients and in the pilocarpine-induced animal model. However, the functional consequences of this alteration for synaptic activity have not yet been fully elucidated. - Source: PubMed
Publication date: 2026/09/11
Nissen Ella MarieKirschstein TimoBertsche AstridKöhling RüdigerFranz Denise - This study aimed to explore the antiepileptic effect of Ganoderic acid A (GA-A) on pentylenetetrazol (PTZ)-induced epilepsy in mice and clarify its underlying mechanism, focusing on the KCNQ2 channel. - Source: PubMed
Yao YangLiu ZhujunWang XujiaoBian CunhaoWang RuiZhang Yukun - Isoform-selective opening of voltage-gated potassium (Kv) channels is desirable for its therapeutic potential yet mechanistically little-understood. Rosemary leaf metabolite carnosic acid (CA) is a high-efficacy opener of the neuronal Kv7.3 channel but exerts minimal effects on Kv7.2 or Kv7.2/3 heteromers. Here, using alanine-scanning mutagenesis, electrophysiology, radioligand binding, and all-atom molecular dynamics simulations, we found that CA achieves high-efficacy activation of Kv7.3 by binding at the extracellular cap of the voltage-sensing domain (VSD), independent of the canonical retigabine pore binding site. Kv7.3 samples a binding-competent VSD conformation absent in Kv7.2, characterized by an expanded extracellular pocket and a favorable electrostatic environment. Central to this mechanism, Kv7.3-L226 functions as a hydrophobic latch that tunes the energetic landscape of the voltage sensor to control ligand efficacy. Unexpectedly, CA potentiates Kv7.2/3 activation by retigabine via ligand-initiated positive allosteric coupling between the VSD and pore, thus enhancing the anticonvulsant action of retigabine despite CA lacking standalone anticonvulsant activity. The findings establish a mechanistic framework in which isoform selectivity arises from VSD conformational microenvironments rather than canonical binding determinants and highlight the voltage sensor as a tunable target for developing selective Kv channel openers. - Source: PubMed
Publication date: 2026/09/23
Manville Rían WFoglia LorenzoYoshimura Ryan FSantos Daniel EYu AlvinAbbott Geoffrey W - To investigate the effectiveness and tolerability of ketogenic diet therapy (KDT) in children with developmental and epileptic encephalopathy (DEE) caused by genetic variants onset within 2 years of age. A retrospective cohort study was conducted. Data were reviewed from 117 children with DEE who started KDT in the Department of Neurology of Beijing Children's Hospital Affiliated to Capital Medical University, Jinan Children's Hospital, Henan Children's Hospital and Urumqi Children's Hospital from January 2016 to June 2023. Data included demographic information, genetic testing results, KDT regimens, and treatment outcomes. Based on the effectiveness of KDT, the children were classified into 4 grades, Grade 1: seizure-free for at least 6 months; Grade 2: 75%-<100% seizure reduction; Grade 3: 50%-<75% seizure reduction; and Grade 4:<50% seizure reduction. The seizure-free group was defined as Grade 1, and the uncontrolled group as Grade 2+3+4; the significantly effective group was defined as Grade 1+2, and the non-responsive group as Grade 3+4. Inter-group comparisons were performed using the test, the independent sample -test, or the Mann-Whitney test. A total of 90 children were enrolled, including 44 males and 46 females, with the age at seizure onset of 2.0 (0.7, 5.3) months. Thirty genes were involved, with ion channels being the most common neurobiological mechanism (49 children (54%)), followed by synaptic function (7 children (8%)). The age at KDT initiation was 11.0 (5.8, 21.8) months, and the time interval between seizure onset and KDT initiation was 8.0 (4.0, 18.3) months. Following KDT, 31 children (34%) achieved Grade 1 (seizure freedom), 19 children (21%) achieved Grade 2, 13 children (14%) achieved Grade 3, and 27 children (30%) achieved Grade 4. There were 24, 9, 9, 6, 5, and 5 children with SCN1A, CDKL5, SCN2A, KCNQ2, STXBP1 and KCNT1 genes variants, respectively; 7, 4, 2, 2, 4, and 1 of these children, respectively, achieved seizure freedom. Among the seizure-free and uncontrolled groups (31 and 59 children), the maintenance lipid-to-nonlipid ratio was (2.2±0.7) and (2.5±0.7):1, 19 children (61%) and 22 children (37%) had non-ion channel gene variants, respectively, both represented statistically difference (both <0.05). Among the significantly effective and non-responsive groups (50 and 40 children), there were 31 females (62%) and 15 females (38%), the interval between seizure onset and KDT initiation was 6.0 (3.8, 12.3) and 10.0 (5.3, 27.5) months, both with statistically difference (both <0.05). Adverse events occurred in 59 children (66%), with gastrointestinal symptoms being the most common (53 children). The main reasons for discontinuing KDT in 73 children were dissatisfaction with efficacy (29 children (40%)) and adverse reactions (14 children (19%)); among them, 2 were due to intolerance resulting from a rapid increase in the lipid-to-nonlipid ratio. KDT is effective in most children with genetic DEE onset within 2 years of age. Earlier initiation yields better results, and seizure freedom is not associated with higher lipid-to-nonlipid ratios. Children with variants in genes encoding non-ion channel proteins, such as STXBP1, respond well to KDT. Most children tolerate KDT well, and a cautious, standardized ketogenic regimen helps to prevent discontinuation due to adverse reactions. - Source: PubMed
Publication date: 2026/09/15
Song T YDeng JChen C HWang X HTian X JWu M LWang XZhang H WChen X YWang YNiyazi YushanjiangAbudureyimu GulixianFang F - There has been considerable interest in developing openers of neuronal K7 potassium channels, particularly K7.2 (KCNQ2) and K7.3 (KCNQ3), as both genetic and clinical evidence strongly support their therapeutic potential in seizure disorders. Herein, we describe the discovery of a series of neuronal K7 potassium channel openers, optimized primarily via conformational rigidification and carbon to oxygen replacement. The combination of these structural modifications, together with careful investigation of the structure-activity relationship (SAR), culminated in the discovery of , a potent and metabolically stable neuronal K7 opener that demonstrated favorable pharmacokinetics (PK) in preclinical species and robust antiseizure efficacy in rodent alternating-current maximal electroshock seizure (AC-MES) assays. - Source: PubMed
Publication date: 2026/08/22
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