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L-type Ca2+ channel-mediated Ca2+ influx adjusts neuronal mitochondrial function to physiological and pathophysiological conditions.

Hotka M, Cagalinec M, Hilber K, Hool L, Boehm S, Kubista H.

Sci Signal. 2020 Feb 11;13(618). pii: eaaw6923. doi: 10.1126/scisignal.aaw6923.


Mitochondria at the Crossroads of Survival and Demise.

Hendgen-Cotta UB, Giorgio V, Hool L.

Oxid Med Cell Longev. 2019 Dec 4;2019:2608187. doi: 10.1155/2019/2608187. eCollection 2019. No abstract available.


Fidelity of translation initiation is required for coordinated respiratory complex assembly.

Rudler DL, Hughes LA, Perks KL, Richman TR, Kuznetsova I, Ermer JA, Abudulai LN, Shearwood AJ, Viola HM, Hool LC, Siira SJ, Rackham O, Filipovska A.

Sci Adv. 2019 Dec 20;5(12):eaay2118. doi: 10.1126/sciadv.aay2118. eCollection 2019 Dec.


Neuronal nitric oxide synthase regulation of calcium cycling in ventricular cardiomyocytes is independent of Cav1.2 channel modulation under basal conditions.

Ebner J, Cagalinec M, Kubista H, Todt H, Szabo PL, Kiss A, Podesser BK, Cserne Szappanos H, Hool LC, Hilber K, Koenig X.

Pflugers Arch. 2020 Jan;472(1):61-74. doi: 10.1007/s00424-019-02335-7. Epub 2019 Dec 10.


Stress signaling and cellular proliferation reverse the effects of mitochondrial mistranslation.

Ferreira N, Perks KL, Rossetti G, Rudler DL, Hughes LA, Ermer JA, Scott LH, Kuznetsova I, Richman TR, Narayana VK, Abudulai LN, Shearwood AJ, Cserne Szappanos H, Tull D, Yeoh GC, Hool LC, Filipovska A, Rackham O.

EMBO J. 2019 Dec 16;38(24):e102155. doi: 10.15252/embj.2019102155. Epub 2019 Nov 13.


TGF-β-induced fibrotic stress increases G-quadruplex formation in human fibroblasts.

Toshniwal P, Nguyen M, Guédin A, Viola H, Ho D, Kim Y, Bhatt U, Bond CS, Hool L, Hurley LH, Mergny JL, Fear M, Wood F, Iyer SK, Smith NM.

FEBS Lett. 2019 Nov;593(22):3149-3161. doi: 10.1002/1873-3468.13658. Epub 2019 Nov 15.


A Review of in vitro Platforms for Understanding Cardiomyocyte Mechanobiology.

Chin IL, Hool L, Choi YS.

Front Bioeng Biotechnol. 2019 Jun 5;7:133. doi: 10.3389/fbioe.2019.00133. eCollection 2019. Review.


Millimeter Wave Radiation Activates Leech Nociceptors via TRPV1-Like Receptor Sensitization.

Romanenko S, Harvey AR, Hool L, Fan S, Wallace VP.

Biophys J. 2019 Jun 18;116(12):2331-2345. doi: 10.1016/j.bpj.2019.04.021. Epub 2019 Apr 25.


Myoglobinopathy is an adult-onset autosomal dominant myopathy with characteristic sarcoplasmic inclusions.

Olivé M, Engvall M, Ravenscroft G, Cabrera-Serrano M, Jiao H, Bortolotti CA, Pignataro M, Lambrughi M, Jiang H, Forrest ARR, Benseny-Cases N, Hofbauer S, Obinger C, Battistuzzi G, Bellei M, Borsari M, Di Rocco G, Viola HM, Hool LC, Cladera J, Lagerstedt-Robinson K, Xiang F, Wredenberg A, Miralles F, Baiges JJ, Malfatti E, Romero NB, Streichenberger N, Vial C, Claeys KG, Straathof CSM, Goris A, Freyer C, Lammens M, Bassez G, Kere J, Clemente P, Sejersen T, Udd B, Vidal N, Ferrer I, Edström L, Wedell A, Laing NG.

Nat Commun. 2019 Mar 27;10(1):1396. doi: 10.1038/s41467-019-09111-2.


Impaired calcium handling and mitochondrial metabolic dysfunction as early markers of hypertrophic cardiomyopathy.

Viola HM, Hool LC.

Arch Biochem Biophys. 2019 Apr 15;665:166-174. doi: 10.1016/ Epub 2019 Mar 16. Review.


Development of induced pluripotent stem cells from a patient with hypertrophic cardiomyopathy who carries the pathogenic myosin heavy chain 7 mutation p.Arg403Gln.

Holliday M, Ross SB, Lim S, Mangala M, Hill A, Szappanos HC, Hool L, Semsarian C.

Stem Cell Res. 2018 Dec;33:269-273. doi: 10.1016/j.scr.2018.11.011. Epub 2018 Nov 28.


A platform for discovery of functional cell-penetrating peptides for efficient multi-cargo intracellular delivery.

Hoffmann K, Milech N, Juraja SM, Cunningham PT, Stone SR, Francis RW, Anastasas M, Hall CM, Heinrich T, Bogdawa HM, Winslow S, Scobie MN, Dewhurst RE, Florez L, Ong F, Kerfoot M, Champain D, Adams AM, Fletcher S, Viola HM, Hool LC, Connor T, Longville BAC, Tan YF, Kroeger K, Morath V, Weiss GA, Skerra A, Hopkins RM, Watt PM.

Sci Rep. 2018 Aug 22;8(1):12538. doi: 10.1038/s41598-018-30790-2.


Concerted regulation of mitochondrial and nuclear non-coding RNAs by a dual-targeted RNase Z.

Siira SJ, Rossetti G, Richman TR, Perks K, Ermer JA, Kuznetsova I, Hughes L, Shearwood AJ, Viola HM, Hool LC, Rackham O, Filipovska A.

EMBO Rep. 2018 Oct;19(10). pii: e46198. doi: 10.15252/embr.201846198. Epub 2018 Aug 20.


A Morpholino Oligomer Therapy Regime That Restores Mitochondrial Function and Prevents mdx Cardiomyopathy.

Viola HM, Johnstone VPA, Adams AM, Fletcher S, Hool LC.

JACC Basic Transl Sci. 2018 Jun 25;3(3):391-402. doi: 10.1016/j.jacbts.2018.03.007. eCollection 2018 Jun.


Author Correction: Identification of a novel cAMP dependent protein kinase A phosphorylation site on the human cardiac calcium channel.

Szappanos HC, Muralidharan P, Ingley E, Petereit J, Millar AH, Hool LC.

Sci Rep. 2018 May 3;8(1):7207. doi: 10.1038/s41598-018-24792-3.


PTCD1 Is Required for 16S rRNA Maturation Complex Stability and Mitochondrial Ribosome Assembly.

Perks KL, Rossetti G, Kuznetsova I, Hughes LA, Ermer JA, Ferreira N, Busch JD, Rudler DL, Spahr H, Schöndorf T, Shearwood AJ, Viola HM, Siira SJ, Hool LC, Milenkovic D, Larsson NG, Rackham O, Filipovska A.

Cell Rep. 2018 Apr 3;23(1):127-142. doi: 10.1016/j.celrep.2018.03.033.


The interaction between electromagnetic fields at megahertz, gigahertz and terahertz frequencies with cells, tissues and organisms: risks and potential.

Romanenko S, Begley R, Harvey AR, Hool L, Wallace VP.

J R Soc Interface. 2017 Dec;14(137). pii: 20170585. doi: 10.1098/rsif.2017.0585. Review.


Frequency-specific effects of repetitive magnetic stimulation on primary astrocyte cultures.

Clarke D, Penrose MA, Penstone T, Fuller-Carter PI, Hool LC, Harvey AR, Rodger J, Bates KA.

Restor Neurol Neurosci. 2017;35(6):557-569. doi: 10.3233/RNN-160708.


Identification of a novel cAMP dependent protein kinase A phosphorylation site on the human cardiac calcium channel.

Cserne Szappanos H, Muralidharan P, Ingley E, Petereit J, Millar AH, Hool LC.

Sci Rep. 2017 Nov 9;7(1):15118. doi: 10.1038/s41598-017-15087-0. Erratum in: Sci Rep. 2018 May 3;8(1):7207.


Real-Time Bioimpedance Sensing of Antifibrotic Drug Action in Primary Human Cells.

Parviz M, Toshniwal P, Viola HM, Hool LC, Fear PMW, Wood FM, Gaus K, Iyer KS, Gooding JJ.

ACS Sens. 2017 Oct 27;2(10):1482-1490. doi: 10.1021/acssensors.7b00442. Epub 2017 Sep 19.


Adult-onset obesity is triggered by impaired mitochondrial gene expression.

Perks KL, Ferreira N, Richman TR, Ermer JA, Kuznetsova I, Shearwood AJ, Lee RG, Viola HM, Johnstone VPA, Matthews V, Hool LC, Rackham O, Filipovska A.

Sci Adv. 2017 Aug 16;3(8):e1700677. doi: 10.1126/sciadv.1700677. eCollection 2017 Aug.


Auto-regulation in the powerhouse.

Viola HM, Hool LC.

Elife. 2017 Jul 10;6. pii: e28757. doi: 10.7554/eLife.28757.


Dystrophic Cardiomyopathy-Potential Role of Calcium in Pathogenesis, Treatment and Novel Therapies.

Johnstone VP, Viola HM, Hool LC.

Genes (Basel). 2017 Mar 24;8(4). pii: E108. doi: 10.3390/genes8040108. Review.


L-type calcium channel: Clarifying the "oxygen sensing hypothesis".

Cserne Szappanos H, Viola H, Hool LC.

Int J Biochem Cell Biol. 2017 May;86:32-36. doi: 10.1016/j.biocel.2017.03.010. Epub 2017 Mar 18. Review.


The cardiac L-type calcium channel alpha subunit is a target for direct redox modification during oxidative stress-the role of cysteine residues in the alpha interacting domain.

Muralidharan P, Cserne Szappanos H, Ingley E, Hool LC.

Clin Exp Pharmacol Physiol. 2017 Dec;44 Suppl 1:46-54. doi: 10.1111/1440-1681.12750. Epub 2017 Sep 20. Review.


Specific combinations of ion channel inhibitors reduce excessive Ca2+ influx as a consequence of oxidative stress and increase neuronal and glial cell viability in vitro.

O'Hare Doig RL, Bartlett CA, Smith NM, Hodgetts SI, Dunlop SA, Hool L, Fitzgerald M.

Neuroscience. 2016 Dec 17;339:450-462. doi: 10.1016/j.neuroscience.2016.10.005. Epub 2016 Oct 8.


The impact of non-severe burn injury on cardiac function and long-term cardiovascular pathology.

O'Halloran E, Shah A, Dembo L, Hool L, Viola H, Grey C, Boyd J, O'Neill T, Wood F, Duke J, Fear M.

Sci Rep. 2016 Oct 3;6:34650. doi: 10.1038/srep34650.


The L-type Ca2+ channel: A mediator of hypertrophic cardiomyopathy.

Viola HM, Hool LC.

Channels (Austin). 2017 Jan 2;11(1):5-7. doi: 10.1080/19336950.2016.1213053. Epub 2016 Jul 20. Review. No abstract available.


Loss of the RNA-binding protein TACO1 causes late-onset mitochondrial dysfunction in mice.

Richman TR, Spåhr H, Ermer JA, Davies SM, Viola HM, Bates KA, Papadimitriou J, Hool LC, Rodger J, Larsson NG, Rackham O, Filipovska A.

Nat Commun. 2016 Jun 20;7:11884. doi: 10.1038/ncomms11884.


The L-type Ca(2+) channel facilitates abnormal metabolic activity in the cTnI-G203S mouse model of hypertrophic cardiomyopathy.

Viola H, Johnstone V, Cserne Szappanos H, Richman T, Tsoutsman T, Filipovska A, Semsarian C, Hool L.

J Physiol. 2016 Jul 15;594(14):4051-70. doi: 10.1113/JP271681. Epub 2016 Jun 12.


The potential for nanotechnology to improve delivery of therapy to the acute ischemic heart.

Evans CW, Iyer KS, Hool LC.

Nanomedicine (Lond). 2016 Apr;11(7):817-32. doi: 10.2217/nnm.16.7. Epub 2016 Mar 16. Review.


Verapamil is Less Effective than Triamcinolone for Prevention of Keloid Scar Recurrence After Excision in a Randomized Controlled Trial.

Danielsen PL, Rea SM, Wood FM, Fear MW, Viola HM, Hool LC, Gankande TU, Alghamdi M, Stevenson AW, Manzur M, Wallace HJ.

Acta Derm Venereol. 2016 Aug 23;96(6):774-8. doi: 10.2340/00015555-2384.


The Role of the L-Type Ca2+ Channel in Altered Metabolic Activity in a Murine Model of Hypertrophic Cardiomyopathy.

Viola HM, Johnstone VPA, Cserne Szappanos H, Richman TR, Tsoutsman T, Filipovska A, Semsarian C, Seidman JG, Seidman CE, Hool LC.

JACC Basic Transl Sci. 2016 Feb 13;1(1-2):61-72. doi: 10.1016/j.jacbts.2015.12.001. eCollection 2016 Jan-Feb. Review.


Evidence for redox sensing by a human cardiac calcium channel.

Muralidharan P, Cserne Szappanos H, Ingley E, Hool L.

Sci Rep. 2016 Jan 11;6:19067. doi: 10.1038/srep19067.


Mutation in MRPS34 compromises protein synthesis and causes mitochondrial dysfunction.

Richman TR, Ermer JA, Davies SM, Perks KL, Viola HM, Shearwood AM, Hool LC, Rackham O, Filipovska A.

PLoS Genet. 2015 Mar 27;11(3):e1005089. doi: 10.1371/journal.pgen.1005089. eCollection 2015 Mar.


Prolonged glutamate excitotoxicity increases GluR1 immunoreactivity but decreases mRNA of GluR1 and associated regulatory proteins in dissociated rat retinae in vitro.

Challenor M, O'Hare Doig R, Fuller P, Giacci M, Bartlett C, Wale CH, Cozens GS, Hool L, Dunlop S, Swaminathan Iyer K, Rodger J, Fitzgerald M.

Biochimie. 2015 May;112:160-71. doi: 10.1016/j.biochi.2015.03.008. Epub 2015 Mar 17.


Deranged sodium to sudden death.

Clancy CE, Chen-Izu Y, Bers DM, Belardinelli L, Boyden PA, Csernoch L, Despa S, Fermini B, Hool LC, Izu L, Kass RS, Lederer WJ, Louch WE, Maack C, Matiazzi A, Qu Z, Rajamani S, Rippinger CM, Sejersted OM, O'Rourke B, Weiss JN, Varró A, Zaza A.

J Physiol. 2015 Mar 15;593(6):1331-45. doi: 10.1113/jphysiol.2014.281204. Review. Erratum in: J Physiol. 2016 Mar 15;594(6):1791.


Nanoparticle-mediated dual delivery of an antioxidant and a peptide against the L-Type Ca2+ channel enables simultaneous reduction of cardiac ischemia-reperfusion injury.

Hardy N, Viola HM, Johnstone VP, Clemons TD, Cserne Szappanos H, Singh R, Smith NM, Iyer KS, Hool LC.

ACS Nano. 2015 Jan 27;9(1):279-89. doi: 10.1021/nn5061404. Epub 2014 Dec 22.


Choline kinase β mutant mice exhibit reduced phosphocholine, elevated osteoclast activity, and low bone mass.

Kular J, Tickner JC, Pavlos NJ, Viola HM, Abel T, Lim BS, Yang X, Chen H, Cook R, Hool LC, Zheng MH, Xu J.

J Biol Chem. 2015 Jan 16;290(3):1729-42. doi: 10.1074/jbc.M114.567966. Epub 2014 Dec 1.


Cellular and molecular changes to cortical neurons following low intensity repetitive magnetic stimulation at different frequencies.

Grehl S, Viola HM, Fuller-Carter PI, Carter KW, Dunlop SA, Hool LC, Sherrard RM, Rodger J.

Brain Stimul. 2015 Jan-Feb;8(1):114-23. doi: 10.1016/j.brs.2014.09.012. Epub 2014 Oct 16.


Glutathionylation of the L-type Ca2+ channel in oxidative stress-induced pathology of the heart.

Johnstone VP, Hool LC.

Int J Mol Sci. 2014 Oct 22;15(10):19203-25. doi: 10.3390/ijms151019203. Review.


Impaired functional communication between the L-type calcium channel and mitochondria contributes to metabolic inhibition in the mdx heart.

Viola HM, Adams AM, Davies SM, Fletcher S, Filipovska A, Hool LC.

Proc Natl Acad Sci U S A. 2014 Jul 15;111(28):E2905-14. doi: 10.1073/pnas.1402544111. Epub 2014 Jun 26.


How does the heart sense changes in oxygen tension: a role for ion channels?

Hool LC.

Antioxid Redox Signal. 2015 Feb 20;22(6):522-36. doi: 10.1089/ars.2014.5880. Epub 2014 May 13. Review.


How does calcium regulate mitochondrial energetics in the heart? - new insights.

Viola HM, Hool LC.

Heart Lung Circ. 2014 Jul;23(7):602-9. doi: 10.1016/j.hlc.2014.02.009. Epub 2014 Feb 28. Review.


Role of the cytoskeleton in communication between L-type Ca(2+) channels and mitochondria.

Viola HM, Hool LC.

Clin Exp Pharmacol Physiol. 2013 Apr;40(4):295-304. doi: 10.1111/1440-1681.12072.


Examining efficacy of "TAT-less" delivery of a peptide against the L-type calcium channel in cardiac ischemia-reperfusion injury.

Clemons TD, Viola HM, House MJ, Iyer KS, Hool LC.

ACS Nano. 2013 Mar 26;7(3):2212-20. doi: 10.1021/nn305211f. Epub 2013 Mar 5.


L-type Ca(2+) channel contributes to alterations in mitochondrial calcium handling in the mdx ventricular myocyte.

Viola HM, Davies SM, Filipovska A, Hool LC.

Am J Physiol Heart Circ Physiol. 2013 Mar 15;304(6):H767-75. doi: 10.1152/ajpheart.00700.2012. Epub 2013 Jan 18.


Regulator of G-protein signaling 5 controls blood pressure homeostasis and vessel wall remodeling.

Holobotovskyy V, Manzur M, Tare M, Burchell J, Bolitho E, Viola H, Hool LC, Arnolda LF, McKitrick DJ, Ganss R.

Circ Res. 2013 Mar 1;112(5):781-91. doi: 10.1161/CIRCRESAHA.111.300142. Epub 2013 Jan 9.


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