Ask about this productRelated genes to: KCNC3 Blocking Peptide
- Gene:
- KCNC3 NIH gene
- Name:
- potassium voltage-gated channel subfamily C member 3
- Previous symbol:
- SCA13
- Synonyms:
- Kv3.3
- Chromosome:
- 19q13.33
- Locus Type:
- gene with protein product
- Date approved:
- 1991-08-13
- Date modifiied:
- 2019-04-23
Related products to: KCNC3 Blocking Peptide
Related articles to: KCNC3 Blocking Peptide
- Kv3.3 voltage-gated K (Kv) channels are highly expressed in cerebellar Purkinje neurons and some hippocampal neurons, aligning with the motor and cognitive impairments observed in spinocerebellar ataxia 13 (SCA13) caused by Kv3.3 mutations. Despite their functional significance, the mechanisms governing Kv3.3 subcellular localization remain poorly understood. Here we report microtubule-associated protein 6 (MAP6) regulates Kv3.3 axon-dendrite targeting. MAP6 deletion reduces Kv3.3 levels in the processes of Purkinje neurons. Mechanistically, MAP6's 1st and 2nd Mn modules directly bind the external surface of the Kv3.3 N-terminal T1 tetramer, while its 3rd Mn module indirectly associates with Cav2 Ca channels. In Purkinje neurons, shRNA-mediated MAP6 knockdown decreases somatodendritic levels of both Kv3.3 and Cav2.1 (associated with SCA6). Notably, expression of Mn1/2-GFP selectively reduces Kv3.3, but not Cav2.1, levels. Purkinje neuron burst firing is reduced in both conditions. These findings uncover a MAP6-dependent mechanism for targeting two key ion channels linked to SCAs. - Source: PubMed
Publication date: 2026/04/30
Ma DiNalinakshan NandithaMarshall Alec HJukkola PeterBosc ChristopheGory-Fauré SylvieAndrieux AnnieWester Jason CGu Chen - An aberrant expression of the voltage-gated potassium (Kv) channel family has been verified in a variety of tumors, which can contribute to tumor proliferation and migration by regulating the cell cycle, suppressing apoptosis, facilitating the epithelial-mesenchymal transition (EMT), and activating downstream tumor-associated pathways. Kv3.3 is encoded by the Kv channel subfamily C member 3 (). Previous studies on focused primarily on nervous system diseases rather than tumors. Therefore, our aim is to explore the potential biological roles and clinical significance of in the field of oncology. - Source: PubMed
Publication date: 2025/12/19
Xia KunZhu Hong-ChaoLai Fang-YeLi Zi-HaoWang WeiWang Nan - This study explores the cell fate reprogrammability of H3K27M-mutant pediatric high-grade gliomas (pHGG) using neuronal transdifferentiation as a potential targeted therapy. We treated the BT245 patient-derived glioma cell line with pharmacological combinations targeting neuronal differentiation pathways and performed bulk RNA sequencing to characterize gene expression patterns driving cell fate transitions. Our findings reveal that the drug combinations induce transcriptomic changes consistent with differentiation towards neuronal phenotypes, including the upregulation of synaptic and dendritic signaling genes and the downregulation of malignant signatures. In comparison, astrocytic differentiation media (DM) and H3K27M knockout (KO) promote residual astrocytic phenotypes, suggesting neuronal transdifferentiation as a more effective strategy for mitigating tumor aggressiveness and progression. Differentially expressed genes such as GRIK1, GRIN1, NRXN3, NRXN1, CALB2, SCGN, SLC32A1, SLC1A2, KCNC3, and neurodevelopmental regulators including WNT7A, DLX6, ERBB4, ARX, BCL11B, SEMA3C, and FGFBP3 were identified as key markers regulating the neuron-like lineage transition. This study demonstrates that pHGGs can be phenotypically redirected toward neuronal-like identities through modulating cell fate differentiation programs. These findings advance the concept of 'differentiation therapy' as a promising intervention to reduce phenotypic plasticity and malignancy in pHGG ecosystems. While these are early in vitro findings, the potential ability to steer and control glioma cells toward stable, less malignant fates offers promising translational potential for patient-centered targeted therapies. - Source: PubMed
Publication date: 2025/12/30
Uthamacumaran AbicumaranHorth CynthiaBareke EricGravel MichelMajewski Jacek - Colorectal cancer (CRC) is a heterogeneous disease that is associated with several genetic or somatic mutations. Cancer immunotherapy has become a novel and revolutionary method of treatment for patients with advanced tumors. However, effective biomarkers that can reflect the response of CRC patients to immunotherapy have still not been identified. Our study aimed to explore the expression and functional role of KCNC3 in CRC. - Source: PubMed
Publication date: 2025/04/27
Li QuanqingLv JiayingDuan HongxiaSun PingpingLu BingShi WenyuCai Yihong - Spinocerebellar ataxia 13 (SCA13) is an autosomal dominant neurological disorder caused by mutations in KCNC3. Our previous studies revealed that KCNC3 (Potassium Voltage-Gated Channel Subfamily C Member 3) mutation R423H results in an early-onset form of SCA13. Previous biological models of SCA13 include zebrafish and Drosophila but no mammalian systems. More recently, mouse models with Kcnc3 mutations presented behavioral abnormalities but without obvious pathological changes in the cerebellum, a hallmark of patients with SCA13. Here, we present a novel transgenic mouse model by bacterial artificial chromosome (BAC) recombineering to express the full-length mouse Kcnc3 expressing the R424H mutation. This BAC-R424H mice exhibited behavioral and pathological changes mimicking the clinical phenotype of the disease. The BAC-R424H mice (homologous to R423H in human) developed early onset clinical symptoms with aberrant gait, tremor, and cerebellar atrophy. Histopathological analysis of the cerebellum in BAC-R424H mice showed progressive Purkinje cell loss and thinning of the molecular cell layer. Additionally, Purkinje cells of BAC-R424H mice showed significantly lower spontaneous firing frequency with a corresponding increase in inter-spike interval compared to that of wild-type mice. Our SCA13 transgenic mice recapitulate both neuropathological and behavioral changes manifested in human SCA13 R423H patients and provide an advantageous approach to understanding the role of voltage-gated potassium channel in cerebellar morphogenesis and function. This mammalian in vivo model will lead to further understanding of the R423H allelic form of SCA13 from the molecular to the behavioral level and serve as a platform for testing potential therapeutic compounds. - Source: PubMed
Publication date: 2025/03/20
Yin JunxiangNick Jerelyn AKhare SwatiKloefkorn Heidi EGao MingWu MichaelWhite JenniferResnick James LAllen Kyle DNick Harry SWaters Michael F