KCNJ8 antibody - N - terminal region (OAAB08559)
- Known as:
- KCNJ8 (anti-) - N - terminal region (OAAB08559)
- Catalog number:
- oaab08559
- Product Quantity:
- USD
- Category:
- -
- Supplier:
- Aviva Systems Biology
- Gene target:
- KCNJ8 antibody - terminal region (OAAB08559)
Related genes to: KCNJ8 antibody - N - terminal region (OAAB08559)
- Gene:
- KCNJ8 NIH gene
- Name:
- potassium voltage-gated channel subfamily J member 8
- Previous symbol:
- -
- Synonyms:
- Kir6.1
- Chromosome:
- 12p12.1
- Locus Type:
- gene with protein product
- Date approved:
- 1995-07-18
- Date modifiied:
- 2016-02-04
Related products to: KCNJ8 antibody - N - terminal region (OAAB08559)
Related articles to: KCNJ8 antibody - N - terminal region (OAAB08559)
- The normal structure and function of inner-ear blood vessels, including the microvascular network of the stria vascularis (SV) within the blood-labyrinth barrier (BLB), are essential for auditory function. Despite this, the genetic and molecular characteristics of cochlear vasculature are largely unexplored. In this study, we used single-cell RNA sequencing to profile endothelial cells (ECs) and pericytes (PCs) from the adult mouse cochlea. We found a distinct genetic profile and a higher angiogenic potential than observed in the blood-brain barrier (BBB). Two subclasses of PCs were identified. Type 1 PCs, with high levels of α-smooth muscle actin (Acta2) and Tagln, are located on pre-/post-capillary zones. Type 2 PCs, characterized by low Tagln and high Kcnj8/Abcc9 levels, are found specifically in capillary regions. In an ex vivo explant model, both subclasses showed tip-like behavior during sprouting. Ligand-receptor analysis indicated active EC-PC communication. This communication is mediated by adhesive signals, gap junctions, and vesicle trafficking. Using dual fluorescent reporter mouse models, we showed for the first time that PCs can exhibit tip-associated phenotypic plasticity with detectable NG2/PECAM-1 overlap at the sprout front. This tip-associated state may occur from existing cells or progenitors within the vascular niche. Our findings define the molecular signature of cochlear vessels and identify PCs as targets to promote vascular regeneration. This could have implications for hearing restoration when cochlear blood flow is compromised. - Source: PubMed
Publication date: 2026/06/04
Wang PingtingZhang YunpeiHou ZhiqiangZhang JinhuiSharma KushalShi Xiaorui - Adenosine triphosphate (ATP)-sensitive potassium cardiac channels (K) are composed of inward rectifying potassium channel (Kir) subunit Kir6.1 or Kir6.2, encoded by KCNJ8 or KCNJ11, and the sulfonylurea receptor SUR2 or SUR1, encoded by ABCC9 or ABCC8. - Source: PubMed
Publication date: 2026/04/16
Hu DanHuang YanRangel-Sandoval CinthiaSánchez-Pastor EnriqueOnetti Carlos GFerrer-Villada TaniaJiang Meng-NanHasdemir CanAkin IbrahimZhou Xiao-BoEl-Battrawy IbrahimCui MengRomano JohnPinheiro MariahAcuña-Ochoa Jose GChen LiangZhuang Le-NanHao Guo-LiangZhan Li-YingJiang HongAntzelevitch CharlesBarajas-Martínez Hector - Vascular mural cells (VMCs) are crucial for vascular stability, and their dysfunction underlies cardiovascular pathologies including atherosclerosis and aortic aneurysms. PINCH proteins are core focal adhesion components mediating integrin signaling, yet their roles in VMC development remain elusive. Here, we generated mice with conditional deletion of both PINCH1 and PINCH2 in Pdgfrb-lineage VMCs, which resulted in perinatal lethality accompanied by severe arterial enlargement, hemorrhage and defective angiogenesis. Mutant VMCs exhibited profound defects in cytoskeletal organization, proliferation, differentiation, adhesion and extracellular matrix assembly. Multi-omics analyses revealed that PINCH deficiency dysregulated phospho-signaling networks, hyperactivating PDGFR/EGFR/AKT/ERK and STAT/NF-κB pathways while impairing integrin-FAK-SRC and cell cycle-associated pathways (p53, p27). RNA-seq demonstrated altered expression of genes enriched in immune response (CD74, Tlr2), cytoskeleton (TUBB3, ACTA2) and VMC differentiation (Rgs5, Kcnj8, ABCC9). Importantly, we identified PINCH1 as a nuclear transcriptional coregulator that directly represses proliferative-inflammatory programs while promoting contractile-adhesive and cytoskeletal organization signatures. The clinical relevance of these findings is underscored by downregulation of PINCH genes in human atherosclerosis and Marfan syndrome aneurysms, with conserved dysregulation of key PINCH targets including CD74 and RGS5. Our work reveals a dual cytoplasmic-nuclear mechanism for PINCH in maintaining vascular homeostasis, providing both mechanistic insights and therapeutic targets for vascular diseases. - Source: PubMed
Publication date: 2026/03/08
Wang ChunxiaoJin YaoXin YuanfengXing QiankeZhu HongmingZou QichengYan JieLuo LinaLiang XingqunSun YunfuLiu Zhongmin - : Cardiac arrhythmias are among the leading causes of sudden cardiac death (SCD). Pathogenic variants in potassium channel genes play a key role in inherited arrhythmia syndromes, yet their contribution in Central Asian populations remains poorly characterized. : We performed targeted next-generation sequencing (NGS) using a 96-gene custom Haloplex panel in 79 Kazakhstani patients with clinically diagnosed arrhythmias, including atrioventricular block, sick sinus syndrome, and atrial fibrillation. Detected variants in potassium channel genes were classified according to ACMG guidelines and correlated with clinical phenotypes. : A total of 52 variants were identified across 11 potassium channel genes. Two likely pathogenic variants ( p.Cys66Gly and p.Arg176Trp) and six variants of uncertain significance (VUS) in , , , and were detected. Two novel previously unreported variants were found in and Patients harboring pathogenic variants commonly presented with early-onset arrhythmias or a positive family history of cardiovascular disease. Carriers of variants exhibited mild QT prolongation and recurrent syncope. : This is the first genetic study of potassium channel gene mutations in Kazakhstani patients with cardiac arrhythmias. The detection of pathogenic and novel variants highlights the clinical utility of integrating genetic testing into diagnostic and management pathways for arrhythmia syndromes. Population-specific genomic data are essential for improving risk stratification, guiding medication safety, and enabling cascade family screening in Central Asia. - Source: PubMed
Publication date: 2026/01/26
Chamoieva AyaulymRakhimova SauleAbilova ZhannurAkhmetova AinurAkilzhanova GulbanuZhalbinova MadinaDaniyarov AssetAkilzhanov KenesMolkenov AskhatKairov UlykbekKuanysheva AnargulShaimardanov NurlanAbdrakhmanov AyanBekbossynova MakhabbatAkilzhanova Ainur - The normal structure and function of inner-ear blood vessels, including the microvascular network of the stria vascularis (SV) within the blood-labyrinth barrier (BLB), are essential for auditory function. Despite this, the genetic and molecular characteristics of cochlear vasculature are largely unexplored. In this study, we used single-cell RNA sequencing to profile endothelial cells (ECs) and pericytes (PCs) from the adult mouse cochlea. We found a distinct genetic profile and a higher angiogenic potential than observed in the blood-brain barrier (BBB). Two subclasses of PCs were identified. Type 1 PCs, with high levels of α-smooth muscle actin () and , are located on pre-/post-capillary zones. Type 2 PCs, characterized by low and high levels, are found specifically in capillary regions. In an ex vivo explant model, both subclasses showed tip-like behavior during sprouting. Ligand-receptor analysis indicated active EC-PC communication. This communication is mediated by adhesive signals, gap junctions, and vesicle trafficking. Using dual fluorescent reporter mouse models, we showed for the first time that PCs can transition into tip cells by co-expressing NG2/PECAM-1 signals. This transition may occur from existing cells or progenitors within the vascular niche. Our findings define the molecular signature of cochlear vessels and identify PCs as targets to promote vascular regeneration. This could have implications for hearing restoration when cochlear blood flow is compromised. - Source: PubMed
Publication date: 2026/01/23
Wang PingtingZhang YunpeiHou ZhiqiangZhang JinhuiShi Xiaorui