Sonia Franciosi

Sonia Franciosi

UNVERIFIED PROFILE

Are you Sonia Franciosi?   Register this Author

Register author
Sonia Franciosi

Sonia Franciosi

Publications by authors named "Sonia Franciosi"

Are you Sonia Franciosi?   Register this Author

41Publications

635Reads

48Profile Views

Initially Unexplained Cardiac Arrest in Children and Adolescents: A National Experience from the Canadian Pediatric Heart Rhythm Network.

Heart Rhythm 2020 Feb 6. Epub 2020 Feb 6.

Children's Heart Centre, Division of Cardiology, Department of Pediatrics, British Columbia's Children's Hospital, 4480 Oak St. 1F Clinic, Vancouver, British Columbia, Canada V6H 3N1. Electronic address:

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.hrthm.2020.01.030DOI Listing
February 2020

Chronotropic incompetence as a risk predictor in children and young adults with catecholaminergic polymorphic ventricular tachycardia.

J Cardiovasc Electrophysiol 2019 Oct 11;30(10):1923-1929. Epub 2019 Jul 11.

Division of Cardiology, Department of Pediatrics, Children's Heart Centre, BC Children's Hospital, University of British Columbia, Vancouver, Canada.

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1111/jce.14043DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6786920PMC
October 2019

Dynamic Electrocardiographic Abnormalities Captured in Timothy Syndrome.

JACC Clin Electrophysiol 2018 11;4(11):1486-1487

Department of Pediatrics, Children's Heart Centre, Division of Cardiology, British Columbia Children's Hospital, Vancouver, British Columbia, Canada. Electronic address:

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.jacep.2018.06.019DOI Listing
November 2018

Polymorphic ventricular tachycardia associated with an episode of reflex syncope: Is this the needle in the haystack?

HeartRhythm Case Rep 2018 Nov 4;4(11):510-513. Epub 2018 Aug 4.

Division of Cardiology, Department of Pediatrics, University of British Columbia, Vancouver, British Columbia, Canada.

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.hrcr.2018.07.013DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6241039PMC
November 2018

Preventing mutant huntingtin proteolysis and intermittent fasting promote autophagy in models of Huntington disease.

Acta Neuropathol Commun 2018 03 6;6(1):16. Epub 2018 Mar 6.

Centre for Molecular Medicine and Therapeutics (CMMT), CFRI, Department of Medical Genetics, University of British Columbia, 950 West 28th Avenue, Vancouver, BC, V5Z 4H4, Canada.

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1186/s40478-018-0518-0DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5839066PMC
March 2018

The Safety and Effectiveness of Flecainide in Children in the Current Era.

Pediatr Cardiol 2017 Dec 24;38(8):1633-1638. Epub 2017 Aug 24.

Children's Heart Centre, British Columbia's Children's Hospital, 1F9, 4480 Oak Street, Vancouver, BC, V6H 3V4, Canada.

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1007/s00246-017-1707-5DOI Listing
December 2017

The role of the autonomic nervous system in arrhythmias and sudden cardiac death.

Auton Neurosci 2017 07 31;205:1-11. Epub 2017 Mar 31.

Department of Pediatrics, University of British Columbia, BC Children's Hospital Heart Center, Room 1F9, 4480 Oak Street, V6H 3V4 Vancouver, BC, Canada. Electronic address:

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.autneu.2017.03.005DOI Listing
July 2017

p35 hemizygosity activates Akt but does not improve motor function in the YAC128 mouse model of Huntington's disease.

Neuroscience 2017 06 6;352:79-87. Epub 2017 Apr 6.

Department of Medical Genetics, Centre for Molecular Medicine and Therapeutics, Child and Family Research Institute, University of British Columbia, Vancouver, British Columbia V5Z 4H4, Canada; Division of Neurology, Department of Medicine, University of British Columbia, Vancouver, British Columbia V5Z 4H4, Canada. Electronic address:

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.neuroscience.2017.03.051DOI Listing
June 2017

Sudden death due to paralysis and synaptic and behavioral deficits when Hip14/Zdhhc17 is deleted in adult mice.

BMC Biol 2016 12 7;14(1):108. Epub 2016 Dec 7.

Centre for Molecular Medicine and Therapeutics, Department of Medical Genetics, Child & Family Research Institute, University of British Columbia (UBC), Vancouver, BC, V5Z 4H4, Canada.

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1186/s12915-016-0333-7DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5142322PMC
December 2016

Caspase-6-Resistant Mutant Huntingtin Does not Rescue the Toxic Effects of Caspase-Cleavable Mutant Huntingtin in vivo.

J Huntingtons Dis 2012 ;1(2):243-60

Centre for Molecular Medicine and Therapeutics, Child and Family Research Institute, Department of Medical Genetics, University of British Columbia, Vancouver, BC, Canada.

View Article

Download full-text PDF

Source
http://dx.doi.org/10.3233/JHD-120038DOI Listing
February 2016

A Huntingtin-based peptide inhibitor of caspase-6 provides protection from mutant Huntingtin-induced motor and behavioral deficits.

Hum Mol Genet 2015 May 23;24(9):2604-14. Epub 2015 Jan 23.

Neuroscience Laboratory, Felsenstein Medical Research Center, Sackler Faculty of Medicine, Tel-Aviv University, Israel and

View Article

Download full-text PDF

Source
https://academic.oup.com/hmg/article-lookup/doi/10.1093/hmg/
Publisher Site
http://dx.doi.org/10.1093/hmg/ddv023DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4383866PMC
May 2015

Partial rescue of some features of Huntington Disease in the genetic absence of caspase-6 in YAC128 mice.

Neurobiol Dis 2015 Apr 9;76:24-36. Epub 2015 Jan 9.

Centre for Molecular Medicine and Therapeutics, Child and Family Research Institute, Department of Medical Genetics, University of British Columbia, Vancouver, British Columbia V5Z 4H4, Canada. Electronic address:

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.nbd.2014.12.030DOI Listing
April 2015

Anti-semaphorin 4D immunotherapy ameliorates neuropathology and some cognitive impairment in the YAC128 mouse model of Huntington disease.

Neurobiol Dis 2015 Apr 3;76:46-56. Epub 2015 Feb 3.

Centre for Molecular Medicine and Therapeutics, Child and Family Research Institute, University of British Columbia, Vancouver, BC V5Z 4H4, Canada. Electronic address:

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.nbd.2015.01.002DOI Listing
April 2015

Postnatal muscle modification by myogenic factors modulates neuropathology and survival in an ALS mouse model.

Nat Commun 2013 ;4:2906

1] Department of Medical Genetics, Centre for Molecular Medicine and Therapeutics, Child and Family Research Institute, University of British Columbia, Vancouver, British Columbia, Canada V5Z 4H4 [2] Division of Neurology, Department of Medicine, Centre for Molecular Medicine and Therapeutics, Child and Family Research Institute, University of British Columbia, Vancouver, British Columbia, Canada V5Z 4H4.

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1038/ncomms3906DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4965267PMC
July 2014

A systematic review and meta-analysis of clinical variables used in Huntington disease research.

Mov Disord 2013 Dec 18;28(14):1987-94. Epub 2013 Oct 18.

Center for Molecular Medicine and Therapeutics and Department of Medical Genetics, Child and Family Research Institute, University of British Columbia, Vancouver, British Columbia, Canada.

View Article

Download full-text PDF

Source
http://doi.wiley.com/10.1002/mds.25663
Publisher Site
http://dx.doi.org/10.1002/mds.25663DOI Listing
December 2013

Memory and synaptic deficits in Hip14/DHHC17 knockout mice.

Proc Natl Acad Sci U S A 2013 Dec 25;110(50):20296-301. Epub 2013 Nov 25.

Department of Psychiatry and Brain Research Centre, University of British Columbia, Vancouver, BC, Canada V6T 1Z3.

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1073/pnas.1222384110DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3864353PMC
December 2013

ABCA1 influences neuroinflammation and neuronal death.

Neurobiol Dis 2013 Jun 30;54:445-55. Epub 2013 Jan 30.

Centre for Molecular Medicine and Therapeutics, University of British Columbia, Vancouver, British Columbia, Canada.

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.nbd.2013.01.018DOI Listing
June 2013

A fully humanized transgenic mouse model of Huntington disease.

Hum Mol Genet 2013 Jan 21;22(1):18-34. Epub 2012 Sep 21.

Centre for Molecular Medicine and Therapeutics, Child and Family Research Institute, University of British Columbia, Vancouver, BC, Canada V5Z 4H4.

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1093/hmg/dds397DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3606012PMC
January 2013

NP03, a novel low-dose lithium formulation, is neuroprotective in the YAC128 mouse model of Huntington disease.

Neurobiol Dis 2012 Dec 10;48(3):282-9. Epub 2012 Jul 10.

Centre for Molecular Medicine and Therapeutics, University of British Columbia, and Child and Family Research Institute, Vancouver, BC, Canada.

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.nbd.2012.06.026DOI Listing
December 2012

Low levels of human HIP14 are sufficient to rescue neuropathological, behavioural, and enzymatic defects due to loss of murine HIP14 in Hip14-/- mice.

PLoS One 2012 23;7(5):e36315. Epub 2012 May 23.

Department of Medical Genetics and Centre for Molecular Medicine and Therapeutics, Child and Family Research Institute, University of British Columbia, Vancouver, British Columbia, Canada.

View Article

Download full-text PDF

Source
http://journals.plos.org/plosone/article?id=10.1371/journal.pone.0036315PLOS
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3359340PMC
October 2012

Marked differences in neurochemistry and aggregates despite similar behavioural and neuropathological features of Huntington disease in the full-length BACHD and YAC128 mice.

Hum Mol Genet 2012 May 9;21(10):2219-32. Epub 2012 Feb 9.

Centre for Molecular Medicine and Therapeutics, University of British Columbia, and Child and Family Research Institute, Vancouver, BC, Canada.

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1093/hmg/dds037DOI Listing
May 2012

Age-dependent neurovascular abnormalities and altered microglial morphology in the YAC128 mouse model of Huntington disease.

Neurobiol Dis 2012 Jan 13;45(1):438-49. Epub 2011 Sep 13.

Centre for Molecular Medicine and Therapeutics and Department of Medical Genetics, Child and Family Research Institute, University of British Columbia, Vancouver, BC, Canada V5Z 4H4.

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.nbd.2011.09.003DOI Listing
January 2012

Natural history of disease in the YAC128 mouse reveals a discrete signature of pathology in Huntington disease.

Neurobiol Dis 2011 Jul 31;43(1):257-65. Epub 2011 Mar 31.

Centre for Molecular Medicine and Therapeutics, Child and Family Research Institute, University of British Columbia, Vancouver, Canada V5Z 4H4.

View Article

Download full-text PDF

Source
http://linkinghub.elsevier.com/retrieve/pii/S096999611100100
Publisher Site
http://dx.doi.org/10.1016/j.nbd.2011.03.018DOI Listing
July 2011

Increased locomotor activity in mice lacking the low-density lipoprotein receptor.

Behav Brain Res 2008 Aug 8;191(2):256-65. Epub 2008 Apr 8.

Department of Psychiatry, Mount Sinai School of Medicine, New York, NY 10029, USA.

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.bbr.2008.03.036DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4662864PMC
August 2008

Pepsin pretreatment allows collagen IV immunostaining of blood vessels in adult mouse brain.

J Neurosci Methods 2007 Jun 25;163(1):76-82. Epub 2007 Feb 25.

Department of Psychiatry, Box 1230, Mount Sinai School of Medicine, One Gustave L. Levy Place, New York, NY 10029, USA.

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.jneumeth.2007.02.020DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1931483PMC
June 2007

IL-8 enhancement of amyloid-beta (Abeta 1-42)-induced expression and production of pro-inflammatory cytokines and COX-2 in cultured human microglia.

J Neuroimmunol 2005 Feb 26;159(1-2):66-74. Epub 2004 Nov 26.

Department of Pharmacology and Therapeutics, University of British Columbia, 2176 Health Sciences Mall, Vancouver, BC, Canada, V6T 1Z3.

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.jneuroim.2004.10.006DOI Listing
February 2005

Minocycline inhibits neuronal death and glial activation induced by beta-amyloid peptide in rat hippocampus.

Glia 2004 Oct;48(1):85-90

Department of Pharmacology and Therapeutics, Faculty of Medicine, University of British Columbia, Vancouver, British Columbia, Canada.

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1002/glia.20051DOI Listing
October 2004

Interferon-gamma acutely induces calcium influx in human microglia.

J Neurosci Res 2002 Sep;69(5):607-13

Department of Pharmacology, University of British Columbia, Vancouver, British Columbia, Canada.

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1002/jnr.10331DOI Listing
September 2002