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  1. Article ; Online: Bridging Personal and Population in Excitability Diseases: Will Studies of Rare Diseases Bring Generalizable Mechanisms From Monogenic Channelopathies?

    Nichols, Colin G / McClenaghan, Conor

    Function (Oxford, England)

    2022  Volume 3, Issue 1, Page(s) zqab072

    MeSH term(s) Humans ; Channelopathies/genetics ; Rare Diseases/genetics ; Mental Disorders
    Language English
    Publishing date 2022-01-04
    Publishing country England
    Document type Journal Article
    ISSN 2633-8823
    ISSN (online) 2633-8823
    DOI 10.1093/function/zqab072
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  2. Article ; Online: Kir6.1 and SUR2B in Cantú syndrome.

    McClenaghan, Conor / Nichols, Colin G

    American journal of physiology. Cell physiology

    2022  Volume 323, Issue 3, Page(s) C920–C935

    Abstract: Kir6.1 and SUR2 are subunits of ATP-sensitive potassium ( ... ...

    Abstract Kir6.1 and SUR2 are subunits of ATP-sensitive potassium (K
    MeSH term(s) Adenosine Triphosphate ; Animals ; Cardiomegaly/genetics ; Humans ; Hypertrichosis/genetics ; KATP Channels/genetics ; Mice ; Osteochondrodysplasias/genetics ; Sulfonylurea Receptors/genetics
    Chemical Substances KATP Channels ; Sulfonylurea Receptors ; Adenosine Triphosphate (8L70Q75FXE)
    Language English
    Publishing date 2022-07-25
    Publishing country United States
    Document type Journal Article ; Review
    ZDB-ID 392098-7
    ISSN 1522-1563 ; 0363-6143
    ISSN (online) 1522-1563
    ISSN 0363-6143
    DOI 10.1152/ajpcell.00154.2022
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  3. Article ; Online: Endogenous currents in HEK 293 cells are inhibited by memantine.

    Harrison, Neil L / Abbott, Geoffrey W / McClenaghan, Conor / Nichols, Colin G / Cabrera-Garcia, David

    Nature chemical biology

    2023  Volume 19, Issue 11, Page(s) 1303–1305

    MeSH term(s) Humans ; Memantine/pharmacology ; HEK293 Cells ; Receptors, N-Methyl-D-Aspartate
    Chemical Substances Memantine (W8O17SJF3T) ; Receptors, N-Methyl-D-Aspartate
    Language English
    Publishing date 2023-10-05
    Publishing country United States
    Document type Letter
    ZDB-ID 2202962-X
    ISSN 1552-4469 ; 1552-4450
    ISSN (online) 1552-4469
    ISSN 1552-4450
    DOI 10.1038/s41589-023-01423-1
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    Zs.A 6251: Show issues Location:
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  4. Article ; Online: Rapid Characterization of the Functional and Pharmacological Consequences of Cantú Syndrome K

    Gao, Jian / McClenaghan, Conor / Matreyek, Kenneth A / Grange, Dorothy K / Nichols, Colin G

    The Journal of pharmacology and experimental therapeutics

    2023  Volume 386, Issue 3, Page(s) 298–309

    Abstract: Gain-of-function of ... ...

    Abstract Gain-of-function of K
    MeSH term(s) Humans ; Glyburide/pharmacology ; Glyburide/metabolism ; Pinacidil/pharmacology ; HEK293 Cells ; KATP Channels/genetics ; KATP Channels/metabolism ; Sulfonylurea Receptors/genetics ; Sulfonylurea Receptors/metabolism ; Mutation ; Cardiomegaly/genetics ; Adenosine Triphosphate/metabolism
    Chemical Substances Glyburide (SX6K58TVWC) ; Pinacidil (7B0ZZH8P2W) ; KATP Channels ; Sulfonylurea Receptors ; Adenosine Triphosphate (8L70Q75FXE)
    Language English
    Publishing date 2023-08-01
    Publishing country United States
    Document type Journal Article ; Research Support, N.I.H., Extramural
    ZDB-ID 3106-9
    ISSN 1521-0103 ; 0022-3565
    ISSN (online) 1521-0103
    ISSN 0022-3565
    DOI 10.1124/jpet.123.001659
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  5. Article ; Online: Zoledronic Acid Blocks Overactive Kir6.1/SUR2-Dependent K

    Scala, Rosa / Maqoud, Fatima / McClenaghan, Conor / Harter, Theresa M / Perrone, Maria Grazia / Scilimati, Antonio / Nichols, Colin G / Tricarico, Domenico

    Cells

    2023  Volume 12, Issue 6

    Abstract: Cantú syndrome (CS) is caused by the gain of function mutations in ... ...

    Abstract Cantú syndrome (CS) is caused by the gain of function mutations in the
    MeSH term(s) Animals ; Mice ; Adenosine Triphosphate ; Disease Models, Animal ; Glyburide/pharmacology ; Muscle, Skeletal/drug effects ; Muscle, Skeletal/metabolism ; Osteoblasts/drug effects ; Osteoblasts/metabolism ; Zoledronic Acid/pharmacology ; KATP Channels/drug effects ; KATP Channels/metabolism ; Sulfonylurea Receptors/drug effects ; Sulfonylurea Receptors/metabolism
    Chemical Substances Adenosine Triphosphate (8L70Q75FXE) ; Glyburide (SX6K58TVWC) ; Zoledronic Acid (6XC1PAD3KF) ; uK-ATP-1 potassium channel ; KATP Channels ; Abcc9 protein, mouse ; Sulfonylurea Receptors
    Language English
    Publishing date 2023-03-17
    Publishing country Switzerland
    Document type Journal Article ; Research Support, Non-U.S. Gov't
    ZDB-ID 2661518-6
    ISSN 2073-4409 ; 2073-4409
    ISSN (online) 2073-4409
    ISSN 2073-4409
    DOI 10.3390/cells12060928
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  6. Article ; Online: Skeletal muscle delimited myopathy and verapamil toxicity in SUR2 mutant mouse models of AIMS.

    McClenaghan, Conor / Mukadam, Maya A / Roeglin, Jacob / Tryon, Robert C / Grabner, Manfred / Dayal, Anamika / Meyer, Gretchen A / Nichols, Colin G

    EMBO molecular medicine

    2023  Volume 15, Issue 6, Page(s) e16883

    Abstract: ABCC9-related intellectual disability and myopathy syndrome (AIMS) arises from loss-of-function (LoF) mutations in the ABCC9 gene, which encodes the SUR2 subunit of ATP-sensitive potassium ( ... ...

    Abstract ABCC9-related intellectual disability and myopathy syndrome (AIMS) arises from loss-of-function (LoF) mutations in the ABCC9 gene, which encodes the SUR2 subunit of ATP-sensitive potassium (K
    MeSH term(s) Animals ; Mice ; Adenosine Triphosphate ; Muscle, Skeletal/metabolism ; Muscular Diseases/chemically induced ; Muscular Diseases/genetics ; Potassium Channels, Inwardly Rectifying/genetics ; Potassium Channels, Inwardly Rectifying/metabolism ; Sulfonylurea Receptors/genetics ; Sulfonylurea Receptors/metabolism ; Verapamil/metabolism
    Chemical Substances Adenosine Triphosphate (8L70Q75FXE) ; Potassium Channels, Inwardly Rectifying ; Sulfonylurea Receptors ; Verapamil (CJ0O37KU29) ; Abcc9 protein, mouse
    Language English
    Publishing date 2023-05-08
    Publishing country England
    Document type Journal Article ; Research Support, N.I.H., Extramural ; Research Support, Non-U.S. Gov't
    ZDB-ID 2467145-9
    ISSN 1757-4684 ; 1757-4676
    ISSN (online) 1757-4684
    ISSN 1757-4676
    DOI 10.15252/emmm.202216883
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    Zs.A 6784: Show issues Location:
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  7. Article ; Online: Isolation of Cardiac and Vascular Smooth Muscle Cells from Adult, Juvenile, Larval and Embryonic Zebrafish for Electrophysiological Studies.

    Singareddy, Soma S / McClenaghan, Conor / Roessler, Helen I / Tryon, Robert / Nichols, Colin G

    Journal of visualized experiments : JoVE

    2022  , Issue 180

    Abstract: Zebrafish have long been used as a model vertebrate organism in cardiovascular research. The technical difficulties of isolating individual cells from the zebrafish cardiovascular tissues have been limiting in studying their electrophysiological ... ...

    Abstract Zebrafish have long been used as a model vertebrate organism in cardiovascular research. The technical difficulties of isolating individual cells from the zebrafish cardiovascular tissues have been limiting in studying their electrophysiological properties. Previous methods have been described for dissection of zebrafish hearts and isolation of ventricular cardiac myocytes. However, the isolation of zebrafish atrial and vascular myocytes for electrophysiological characterization was not detailed. This work describes new and modified enzymatic protocols that routinely provide isolated juvenile and adult zebrafish ventricular and atrial cardiomyocytes, as well as vascular smooth muscle (VSM) cells from the bulbous arteriosus, suitable for patch-clamp experiments. There has been no literary evidence of electrophysiological studies on zebrafish cardiovascular tissues isolated at embryonic and larval stages of development. Partial dissociation techniques that allow patch-clamp experiments on individual cells from larval and embryonic hearts are demonstrated.
    MeSH term(s) Animals ; Heart Ventricles ; Larva ; Muscle, Smooth, Vascular ; Myocytes, Cardiac/physiology ; Zebrafish
    Language English
    Publishing date 2022-02-09
    Publishing country United States
    Document type Journal Article ; Research Support, N.I.H., Extramural ; Video-Audio Media
    ZDB-ID 2259946-0
    ISSN 1940-087X ; 1940-087X
    ISSN (online) 1940-087X
    ISSN 1940-087X
    DOI 10.3791/63225
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  8. Article ; Online: Pulmonary Hypertension and ATP-Sensitive Potassium Channels.

    McClenaghan, Conor / Woo, Kel Vin / Nichols, Colin G

    Hypertension (Dallas, Tex. : 1979)

    2019  Volume 74, Issue 1, Page(s) 14–22

    MeSH term(s) Disease Progression ; Female ; Gene Expression Regulation ; Humans ; Hypertension, Pulmonary/drug therapy ; Hypertension, Pulmonary/genetics ; Hypertension, Pulmonary/physiopathology ; KATP Channels/genetics ; Male ; Mutation ; Potassium Channel Blockers/therapeutic use ; Prognosis ; Risk Assessment ; Sulfonylurea Receptors/genetics ; Survival Analysis
    Chemical Substances ABCC8 protein, human ; KATP Channels ; Potassium Channel Blockers ; Sulfonylurea Receptors
    Language English
    Publishing date 2019-05-28
    Publishing country United States
    Document type Journal Article ; Research Support, N.I.H., Extramural ; Research Support, Non-U.S. Gov't ; Review
    ZDB-ID 423736-5
    ISSN 1524-4563 ; 0194-911X ; 0362-4323
    ISSN (online) 1524-4563
    ISSN 0194-911X ; 0362-4323
    DOI 10.1161/HYPERTENSIONAHA.119.12992
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    Zs.A 1488: Show issues Location:
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  9. Article ; Online: Zoledronic Acid Blocks Overactive Kir6.1/SUR2-Dependent K ATP Channels in Skeletal Muscle and Osteoblasts in a Murine Model of Cantú Syndrome

    Rosa Scala / Fatima Maqoud / Conor McClenaghan / Theresa M. Harter / Maria Grazia Perrone / Antonio Scilimati / Colin G. Nichols / Domenico Tricarico

    Cells, Vol 12, Iss 928, p

    2023  Volume 928

    Abstract: Cantú syndrome (CS) is caused by the gain of function mutations in the ABCC9 and KCNJ8 genes encoding, respectively, for the sulfonylureas receptor type 2 (SUR2) and the inwardly rectifier potassium channel 6.1 (Kir6.1) of the ATP-sensitive potassium ( ... ...

    Abstract Cantú syndrome (CS) is caused by the gain of function mutations in the ABCC9 and KCNJ8 genes encoding, respectively, for the sulfonylureas receptor type 2 (SUR2) and the inwardly rectifier potassium channel 6.1 (Kir6.1) of the ATP-sensitive potassium (KATP) channels. CS is a multi-organ condition with a cardiovascular phenotype, neuromuscular symptoms, and skeletal malformations. Glibenclamide has been proposed for use in CS, but even in animals, the drug is incompletely effective against severe mutations, including the Kir6.1 wt/V65M . Patch-clamp experiments showed that zoledronic acid (ZOL) fully reduced the whole-cell KATP currents in bone calvaria cells from wild type (WT/WT) and heterozygous Kir6.1 wt/V65M CS mice, with IC 50 for ZOL block < 1 nM in each case. ZOL fully reduced KATP current in excised patches in skeletal muscle fibers in WT/WT and CS mice, with IC 50 of 100 nM in each case. Interestingly, KATP currents in the bone of heterozygous SUR2 wt/A478V mice were less sensitive to ZOL inhibition, showing an IC 50 of ~500 nM and a slope of ~0.3. In homozygous SUR2 A478V/A478V cells, ZOL failed to fully inhibit the KATP currents, causing only ~35% inhibition at 100 μM, but was responsive to glibenclamide. ZOL reduced the KATP currents in Kir6.1 wt/VM CS mice in both skeletal muscle and bone cells but was not effective in the SUR2 [A478V] mice fibers. These data indicate a subunit specificity of ZOL action that is important for appropriate CS therapies.
    Keywords Cantú syndrome ; rare disease ; ATP-sensitive potassium channel ; skeletal muscle ; glibenclamide ; patch clamp ; Biology (General) ; QH301-705.5
    Subject code 333 ; 572
    Language English
    Publishing date 2023-03-01T00:00:00Z
    Publisher MDPI AG
    Document type Article ; Online
    Database BASE - Bielefeld Academic Search Engine (life sciences selection)

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  10. Article ; Online: Coronavirus Proteins as Ion Channels: Current and Potential Research.

    McClenaghan, Conor / Hanson, Alex / Lee, Sun-Joo / Nichols, Colin G

    Frontiers in immunology

    2020  Volume 11, Page(s) 573339

    Abstract: Coronavirus (CoV) outbreaks have recently emerged as a global public health threat due to their exceptional zoonotic potential - a feature arising from their ability to infect a diverse range of potential hosts combined with their high capacity for ... ...

    Abstract Coronavirus (CoV) outbreaks have recently emerged as a global public health threat due to their exceptional zoonotic potential - a feature arising from their ability to infect a diverse range of potential hosts combined with their high capacity for mutation and recombination. After Severe Acute Respiratory Syndrome (SARS) CoV-1 in 2003 and Middle East Respiratory Syndrome (MERS) CoV in 2012, with the current SARS-CoV-2 pandemic we are now in the midst of the third deadly international CoV outbreak in less than 20 years. Coronavirus outbreaks present a critical threat to global public health and an urgent necessity for therapeutic options. Here, we critically examine the current evidence for ion channel activity in CoV proteins and the potential for modulation as a therapeutic approach.
    MeSH term(s) Animals ; Humans ; Ion Channels/genetics ; Ion Channels/metabolism ; SARS Virus/genetics ; SARS Virus/metabolism ; Severe Acute Respiratory Syndrome/virology ; Viral Envelope Proteins/genetics ; Viral Envelope Proteins/metabolism ; Viral Proteins/genetics ; Viral Proteins/metabolism ; Viroporin Proteins/genetics ; Viroporin Proteins/metabolism
    Chemical Substances 3a protein, SARS-CoV ; E protein, SARS coronavirus ; Ion Channels ; Viral Envelope Proteins ; Viral Proteins ; Viroporin Proteins
    Keywords covid19
    Language English
    Publishing date 2020-10-09
    Publishing country Switzerland
    Document type Journal Article ; Research Support, N.I.H., Extramural ; Review
    ZDB-ID 2606827-8
    ISSN 1664-3224 ; 1664-3224
    ISSN (online) 1664-3224
    ISSN 1664-3224
    DOI 10.3389/fimmu.2020.573339
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