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KIR4.1

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https://www.readbyqxmd.com/read/29722316/astrocytic-kir4-1-potassium-channels-as-a-novel-therapeutic-target-for-epilepsy-and-mood-disorders
#1
Yukihiro Ohno
No abstract text is available yet for this article.
April 2018: Neural Regeneration Research
https://www.readbyqxmd.com/read/29722015/east-sesame-syndrome-review-of-the-literature-and-introduction-of-four-new-latvian-patients
#2
REVIEW
C Marta, M Ieva, I Inna, A Mareta, K Sandra, J Pereca, S Janis, P Dita, S Jurgis
EAST (Epilepsy, Ataxia, Sensorineural deafness, Tubulopathy) or SeSAME (Seizures, Sensorineural deafness, Ataxia, Mental retardation, and Electrolyte imbalance) syndrome is a rare autosomal recessive syndrome first described in 2009 independently by Bockenhauer and Scholl. It is caused by mutations in KCNJ10, which encodes Kir4.1, an inwardly rectifying K+ channel found in the brain, inner ear, kidney and eye. To date, 16 mutations in at least 28 patients have been reported. In this paper, we review mutations causing EAST/SeSAME syndrome, clinical manifestations in detail, and efficacy of treatment in previously reported patients...
May 3, 2018: Clinical Genetics
https://www.readbyqxmd.com/read/29667910/role-of-wnk4-and-kidney-specific-wnk1-in-mediating-the-effect-of-high-dietary-k-intake-on-romk-channel-in-the-distal-convoluted-tubule
#3
Peng Wu, Zhong-Xiuzi Gao, Xiao-Tong Su, David H Ellison, Juliette Hadchouel, Jacques Teulon, Wen-Hui Wang
With-no-lysine kinase 4 (WNK4) and kidney-Specific (KS)-WNK1 regulate ROMK (Kir1.1) channels in a variety of cell models. We now explore the role of WNK4 and KS-WNK1 in regulating ROMK in the native distal convoluted tubule (DCT)/connecting tubule (CNT) by measuring TPNQ (ROMK inhibitor)-sensitive K+ currents with whole-cell recording. TPNQ-sensitive K+ currents in DCT2/CNT of KS-WNK1-/- and WNK4-/- mice were significantly smaller than that of WT mice. In contrast, the basolateral K+ channels (a Kir4.1/5.1 heterotetramer) in the DCT were not inhibited...
April 18, 2018: American Journal of Physiology. Renal Physiology
https://www.readbyqxmd.com/read/29666984/unusual-white-matter-involvement-in-east-syndrome-associated-with-novel-kcnj10-mutations
#4
Mariasavina Severino, Susanna Lualdi, Chiara Fiorillo, Pasquale Striano, Teresa De Toni, Silvio Peluso, Giuseppe De Michele, Andrea Rossi, Mirella Filocamo, Claudio Bruno
BACKGROUND: Epilepsy, ataxia, sensorineural deafness, and tubulopathy (EAST syndrome) is a rare channelopathy due to KCNJ10 mutations. So far, only mild cerebellar hypoplasia and/or dentate nuclei abnormalities have been reported as major neuroimaging findings in these patients. METHODS: We analyzed the clinical and brain MRI features of two unrelated patients (aged 27 and 23 years) with EAST syndrome carrying novel homozygous frameshift mutations (p.Asn232Glnfs*14and p...
April 17, 2018: Journal of Neurology
https://www.readbyqxmd.com/read/29650538/kir-channel-blockages-by-proflavine-derivatives-via-multiple-modes-of-interaction
#5
Atsushi Inanobe, Hideaki Itamochi, Yoshihisa Kurachi
Many compounds inhibit tetrameric and pseudo-tetrameric cation channels by associating with the central cavity located in the middle of the membrane plane. They traverse the ion conduction pathway from intracellular side and access to the cavity. Previously we reported that the bacteriostatic agent, proflavine, preferentially blocked a subset of Kir channels. However, the development of the inhibition of Kir1.1 by the compound was obviously different from that operating in Kir3.2 as a pore blocker. To gain mechanistic insights into the compound-channel interaction, we analyzed its chemical specificity, subunit selectivity, and voltage dependency using 13 different combinations of Kir-channel family members and 11 proflavine derivatives...
April 12, 2018: Molecular Pharmacology
https://www.readbyqxmd.com/read/29625493/characterization-of-a-spontaneously-immortalized-murine-m%C3%A3-ller-glial-cell-line-qmmuc-1
#6
Josy Augustine, Sofia Pavlou, Michael O'Hare, Kevin Harkin, Alan Stitt, Tim Curtis, Heping Xu, Mei Chen
Purpose: Müller glia are critical for the survival of retinal neurons and the integrity of retinal blood vessels. Müller glial cultures are important tools for investigating Müller glial pathophysiology. Here, we report a spontaneously immortalized Müller glial cell line originally cultured and subsequently cloned from mouse pups. The cell line, Queen's University Murine Müller glia Clone-1 (QMMuC-1), has been cultured for over 60 passages, has morphologic features like primary Müller cell (PMC) cultures and remains stable...
March 1, 2018: Investigative Ophthalmology & Visual Science
https://www.readbyqxmd.com/read/29615871/a-novel-kcnj2-mutation-identified-in-an-autistic-proband-affects-the-single-channel-properties-of-kir2-1
#7
Anna Binda, Ilaria Rivolta, Chiara Villa, Elisa Chisci, Massimiliano Beghi, Cesare M Cornaggia, Roberto Giovannoni, Romina Combi
Inwardly rectifying potassium channels (Kir) have been historically associated to several cardiovascular disorders. In particular, loss-of-function mutations in the Kir2.1 channel have been reported in cases affected by Andersen-Tawil syndrome while gain-of-function mutations in the same channel cause the short QT3 syndrome. Recently, a missense mutation in Kir2.1, as well as mutations in the Kir4.1, were reported to be involved in autism spectrum disorders (ASDs) suggesting a role of potassium channels in these diseases and introducing the idea of the existence of K+ channel ASDs...
2018: Frontiers in Cellular Neuroscience
https://www.readbyqxmd.com/read/29606582/kir4-1-dependent-astrocyte-fast-motor-neuron-interactions-are-required-for-peak-strength
#8
Kevin W Kelley, Lucile Ben Haim, Lucas Schirmer, Giulia E Tyzack, Michaela Tolman, John G Miller, Hui-Hsin Tsai, Sandra M Chang, Anna V Molofsky, Yongjie Yang, Rickie Patani, Andras Lakatos, Erik M Ullian, David H Rowitch
Diversified neurons are essential for sensorimotor function, but whether astrocytes become specialized to optimize circuit performance remains unclear. Large fast α-motor neurons (FαMNs) of spinal cord innervate fast-twitch muscles that generate peak strength. We report that ventral horn astrocytes express the inward-rectifying K+ channel Kir4.1 (a.k.a. Kcnj10) around MNs in a VGLUT1-dependent manner. Loss of astrocyte-encoded Kir4.1 selectively altered FαMN size and function and led to reduced peak strength...
April 18, 2018: Neuron
https://www.readbyqxmd.com/read/29590095/astrocytic-kir4-1-channels-and-gap-junctions-account-for-spontaneous-epileptic-seizure
#9
Mengmeng Du, Jiajia Li, Liang Chen, Yuguo Yu, Ying Wu
Experimental recordings in hippocampal slices indicate that astrocytic dysfunction may cause neuronal hyper-excitation or seizures. Considering that astrocytes play important roles in mediating local uptake and spatial buffering of K+ in the extracellular space of the cortical circuit, we constructed a novel model of an astrocyte-neuron network module consisting of a single compartment neuron and 4 surrounding connected astrocytes and including extracellular potassium dynamics. Next, we developed a new model function for the astrocyte gap junctions, connecting two astrocyte-neuron network modules...
March 28, 2018: PLoS Computational Biology
https://www.readbyqxmd.com/read/29581290/osr1-regulates-a-subset-of-inward-rectifier-potassium-channels-via-a-binding-motif-variant
#10
Clinton A Taylor, Sung-Wan An, Sachith Gallolu Kankanamalage, Steve Stippec, Svetlana Earnest, Ashesh T Trivedi, Jonathan Zijiang Yang, Hamid Mirzaei, Chou-Long Huang, Melanie H Cobb
The with-no-lysine (K) (WNK) signaling pathway to STE20/SPS1-related proline- and alanine-rich kinase (SPAK) and oxidative stress-responsive 1 (OSR1) kinase is an important mediator of cell volume and ion transport. SPAK and OSR1 associate with upstream kinases WNK 1-4, substrates, and other proteins through their C-terminal domains which interact with linear R-F-x-V/I sequence motifs. In this study we find that SPAK and OSR1 also interact with similar affinity with a motif variant, R-x-F-x-V/I. Eight of 16 human inward rectifier K+ channels have an R-x-F-x-V motif...
April 10, 2018: Proceedings of the National Academy of Sciences of the United States of America
https://www.readbyqxmd.com/read/29513816/effects-of-aquaporin-4-and-inward-rectifier-potassium-channel-4-1-on-medullospinal-edema-after-methylprednisolone-treatment-to-suppress-acute-spinal-cord-injury-in-rats
#11
Ye Li, Haifeng Hu, Jingchen Liu, Qingsan Zhu, Rui Gu
PURPOSE: To investigate the effects of aquaporin 4 (AQP4) and inward rectifier potassium channel 4.1 (Kir4.1) on medullospinal edema after treatment with methylprednisolone (MP) to suppress acute spinal cord injury (ASCI) in rats. METHODS: Sprague Dawley rats were randomly divided into control, sham, ASCI, and MP-treated ASCI groups. After the induction of ASCI, we injected 30 mg/kg MP via the tail vein at various time points. The Tarlov scoring method was applied to evaluate neurological symptoms, and the wet-dry weights method was applied to measure the water content of the spinal cord...
February 2018: Acta Cirúrgica Brasileira
https://www.readbyqxmd.com/read/29503609/it-s-all-about-timing-the-involvement-of-kir4-1-channel-regulation-in-acute-ischemic-stroke-pathology
#12
REVIEW
Meagan Milton, Patrice D Smith
An acute ischemic stroke is characterized by the presence of a blood clot that limits blood flow to the brain resulting in subsequent neuronal loss. Acute stroke threatens neuronal survival, which relies heavily upon proper function of astrocytes. Neurons are more susceptible to cell death when an astrocyte is unable to carry out its normal functions in supporting the neuron in the area affected by the stroke (Rossi et al., 2007; Takano et al., 2009). For example, under normal conditions, astrocytes initially swell in response to changes in extracellular osmotic pressure and then reduce their regulatory volume in response to volume-activated potassium (K+ ) and chloride channels (Vella et al...
2018: Frontiers in Cellular Neuroscience
https://www.readbyqxmd.com/read/29483225/at2r-angiotensin-ii-type-2-receptor-mediated-regulation-of-ncc-na-cl-cotransporter-and-renal-k-excretion-depends-on-the-k-channel-kir4-1
#13
Peng Wu, Zhong-Xiuzi Gao, Xin-Peng Duan, Xiao-Tong Su, Ming-Xiao Wang, Dao-Hong Lin, Ruimin Gu, Wen-Hui Wang
AT2R (AngII [angiotensin II] type 2 receptor) is expressed in the distal nephrons. The aim of the present study is to examine whether AT2R regulates NCC (Na-Cl cotransporter) and Kir4.1 of the distal convoluted tubule. AngII inhibited the basolateral 40 pS K channel (a Kir4.1/5.1 heterotetramer) in the distal convoluted tubule treated with losartan but not with PD123319. AT2R agonist also inhibits the K channel, indicating that AT2R was involved in tonic regulation of Kir4.1. The infusion of PD123319 stimulated the expression of tNCC (total NCC) and pNCC (phosphorylated NCC; Thr53 ) by a time-dependent way with the peak at 4 days...
April 2018: Hypertension
https://www.readbyqxmd.com/read/29476442/novel-homozygous-kcnj10-mutation-in-a-patient-with-non-syndromic-early-onset-cerebellar-ataxia
#14
Francesco Nicita, Giorgio Tasca, Marta Nardella, Emanuele Bellacchio, Ilaria Camponeschi, Gessica Vasco, Tommaso Schirinzi, Enrico Bertini, Ginevra Zanni
Mutations in KCNJ10, which encodes the inwardly rectifying potassium channel Kir4.1, a primary regulator of membrane excitability and potassium homeostasis, cause a complex syndrome characterized by seizures, sensorineural deafness, ataxia, intellectual disability, and electrolyte imbalance called SeSAME/EAST syndrome. We describe a 41-year-old patient with non-syndromic, slowly progressive, early-onset ataxia. Targeted next-generation sequencing identified a novel c.180 T > G (p.Ile60Met) missense homozygous mutation...
February 23, 2018: Cerebellum
https://www.readbyqxmd.com/read/29474462/advanced-glycation-end-age-product-modification-of-laminin-downregulates-kir4-1-in-retinal-m%C3%A3-ller-cells
#15
Kayla Thompson, Jonathan Chen, Qianyi Luo, Yucheng Xiao, Theodore R Cummins, Ashay D Bhatwadekar
Diabetic retinopathy (DR) is a major cause of adult blindness. Retinal Müller cells maintain water homeostasis and potassium concentration via inwardly rectifying Kir4.1 channels. Accumulation of advanced glycation end products (AGEs) is a major pathologic event in DR. While diabetes leads to a decrease in the Kir4.1 channels, it remains unknown whether AGEs-linked to the basement membrane (BM) affect normal Kir4.1 channels. For this study, we hypothesized that AGE-modification of laminin is detrimental to Kir4...
2018: PloS One
https://www.readbyqxmd.com/read/29464197/mecp2-deficiency-leads-to-loss-of-glial-kir4-1
#16
Uri Kahanovitch, Vishnu A Cuddapah, Natasha L Pacheco, Leanne M Holt, Daniel K Mulkey, Alan K Percy, Michelle L Olsen
Rett syndrome (RTT) is an X-linked neurodevelopmental disorder usually caused by mutations in methyl-CpG-binding protein 2 (MeCP2). RTT is typified by apparently normal development until 6-18 mo of age, when motor and communicative skills regress and hand stereotypies, autonomic symptoms, and seizures present. Restoration of MeCP2 function selectively to astrocytes reversed several deficits in a murine model of RTT, but the mechanism of this rescue is unknown. Astrocytes carry out many essential functions required for normal brain functioning, including extracellular K+ buffering...
January 2018: ENeuro
https://www.readbyqxmd.com/read/29446379/astroglial-kir4-1-in-the-lateral-habenula-drives-neuronal-bursts-in-depression
#17
Yihui Cui, Yan Yang, Zheyi Ni, Yiyan Dong, Guohong Cai, Alexandre Foncelle, Shuangshuang Ma, Kangning Sang, Siyang Tang, Yuezhou Li, Ying Shen, Hugues Berry, Shengxi Wu, Hailan Hu
Enhanced bursting activity of neurons in the lateral habenula (LHb) is essential in driving depression-like behaviours, but the cause of this increase has been unknown. Here, using a high-throughput quantitative proteomic screen, we show that an astroglial potassium channel (Kir4.1) is upregulated in the LHb in rat models of depression. Kir4.1 in the LHb shows a distinct pattern of expression on astrocytic membrane processes that wrap tightly around the neuronal soma. Electrophysiology and modelling data show that the level of Kir4...
February 14, 2018: Nature
https://www.readbyqxmd.com/read/29355592/down-regulation-of-inwardly-rectifying-k-currents-in-astrocytes-derived-from-patients-with-monge-s-disease
#18
Wei Wu, Hang Yao, Helen W Zhao, Juan Wang, Gabriel G Haddad
Chronic mountain sickness (CMS) or Monge's disease is a disease in highlanders. These patients have a variety of neurologic symptoms such as migraine, mental fatigue, confusion, dizziness, loss of appetite, memory loss and neuronal degeneration. The cellular and molecular mechanisms underlying CMS neuropathology is not understood. In the previous study, we demonstrated that neurons derived from CMS patients' fibroblasts have a decreased expression and altered gating properties of voltage-gated sodium channel...
March 15, 2018: Neuroscience
https://www.readbyqxmd.com/read/29344503/genetics-of-magnesium-disorders
#19
REVIEW
Heng Li, Shiren Sun, Jianghua Chen, Goushuang Xu, Hanmin Wang, Qi Qian
Background: Magnesium (Mg2+), the second most abundant cation in the cell, is woven into a multitude of cellular functions. Dysmagnesemia is associated with multiple diseases and, when severe, can be life-threatening. Summary: This review discusses Mg2+ homeostasis and function with specific focus on renal Mg2+ handling. Intrarenal channels and transporters related to Mg2+ absorption are discussed. Unraveling the rare genetic diseases with manifestations of dysmagnesemia has greatly increased our understanding of the complex and intricate regulatory network in the kidney, specifically, functions of tight junction proteins including claudin-14, -16, -19, and -10; apical ion channels including: TRPM6, Kv1...
December 2017: Kidney Diseases
https://www.readbyqxmd.com/read/29310825/potassium-intake-modulates-the-thiazide-sensitive-sodium-chloride-cotransporter-ncc-activity-via-the-kir4-1-potassium-channel
#20
Ming-Xiao Wang, Catherina A Cuevas, Xiao-Tong Su, Peng Wu, Zhong-Xiuzi Gao, Dao-Hong Lin, James A McCormick, Chao-Ling Yang, Wen-Hui Wang, David H Ellison
Kir4.1 in the distal convoluted tubule plays a key role in sensing plasma potassium and in modulating the thiazide-sensitive sodium-chloride cotransporter (NCC). Here we tested whether dietary potassium intake modulates Kir4.1 and whether this is essential for mediating the effect of potassium diet on NCC. High potassium intake inhibited the basolateral 40 pS potassium channel (a Kir4.1/5.1 heterotetramer) in the distal convoluted tubule, decreased basolateral potassium conductance, and depolarized the distal convoluted tubule membrane in Kcnj10flox/flox mice, herein referred to as control mice...
April 2018: Kidney International
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