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Items: 1 to 20 of 34


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.


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.


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.


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.


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.


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.


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.


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.


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.


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