Publications by authors named "Tania F Gendron"

78Publications

Chimeric Peptide Species Contribute to Divergent Dipeptide Repeat Pathology in c9ALS/FTD and SCA36.

Neuron 2020 07 5;107(2):292-305.e6. Epub 2020 May 5.

Department of Cell Biology, Emory University, Atlanta, GA 30322, USA; Laboratory for Translational Cell Biology, Emory University, Atlanta, GA 30322, USA; Wallace H. Coulter Graduate Program in Biomedical Engineering, Georgia Institute of Technology & Emory University, Atlanta, GA 30332, USA; Department of Neurology, Emory University, Atlanta, GA 30322, USA. Electronic address:

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http://dx.doi.org/10.1016/j.neuron.2020.04.011DOI Listing
July 2020

Dipeptide repeat proteins inhibit homology-directed DNA double strand break repair in C9ORF72 ALS/FTD.

Mol Neurodegener 2020 02 24;15(1):13. Epub 2020 Feb 24.

Department of Psychiatry & Behavioral Sciences, Center for Therapeutic Innovation, University of Miami Miller School of Medicine, 1501 NW 10th Ave, Biomedical Research Building Room 413, Florida, Miami, 33136, USA.

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http://dx.doi.org/10.1186/s13024-020-00365-9DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7041170PMC
February 2020

Nucleocytoplasmic Proteomic Analysis Uncovers eRF1 and Nonsense-Mediated Decay as Modifiers of ALS/FTD C9orf72 Toxicity.

Neuron 2020 04 13;106(1):90-107.e13. Epub 2020 Feb 13.

The Ken & Ruth Davee Department of Neurology, Northwestern University Feinberg School of Medicine, Chicago, IL 60611, USA; Department of Physiology, Northwestern University Feinberg School of Medicine, Chicago, IL 60611, USA. Electronic address:

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http://dx.doi.org/10.1016/j.neuron.2020.01.020DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7272217PMC
April 2020

Transcription elongation factor AFF2/FMR2 regulates expression of expanded GGGGCC repeat-containing C9ORF72 allele in ALS/FTD.

Nat Commun 2019 11 29;10(1):5466. Epub 2019 Nov 29.

Department of Neurology, University of Massachusetts Medical School, Worcester, MA, 01605, USA.

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http://dx.doi.org/10.1038/s41467-019-13477-8DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6884579PMC
November 2019

Biomarkers for Amyotrophic Lateral Sclerosis and Frontotemporal Dementia Associated With Hexanucleotide Expansion Mutations in .

Front Neurol 2018 5;9:1063. Epub 2018 Dec 5.

Department of Neuroscience, Mayo Clinic, Jacksonville, FL, United States.

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http://dx.doi.org/10.3389/fneur.2018.01063DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6289985PMC
December 2018

Amyotrophic Lateral Sclerosis: An Update for 2018.

Mayo Clin Proc 2018 11 4;93(11):1617-1628. Epub 2018 Jul 4.

Department of Neurology, Mayo Clinic, Rochester, MN.

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https://linkinghub.elsevier.com/retrieve/pii/S00256196183026
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http://dx.doi.org/10.1016/j.mayocp.2018.04.007DOI Listing
November 2018

OPTN p.Met468Arg and ATXN2 intermediate length polyQ extension in families with C9orf72 mediated amyotrophic lateral sclerosis and frontotemporal dementia.

Am J Med Genet B Neuropsychiatr Genet 2018 Jan 28;177(1):75-85. Epub 2017 Oct 28.

Robarts Research Institute, Schulich School of Medicine and Dentistry, Western University, London, Ontario, Canada.

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http://dx.doi.org/10.1002/ajmg.b.32606DOI Listing
January 2018

ARHGEF28 p.Lys280Metfs40Ter in an amyotrophic lateral sclerosis family with a C9orf72 expansion.

Neurol Genet 2017 Oct 22;3(5):e190. Epub 2017 Sep 22.

Department of Biochemistry (S.M.K.F., R.A.H.), Robarts Research Institute, Schulich School of Medicine and Dentistry, Western University, London, Ontario, Canada; Department of Neuroscience (T.F.G., L.P.), Mayo Clinic, Jacksonville, FL; and Department of Clinical Neurological Sciences (M.J.S.), Robarts Research Institute, Schulich School of Medicine and Dentistry, Western University, London, Ontario, Canada.

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http://dx.doi.org/10.1212/NXG.0000000000000190DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5616022PMC
October 2017

Abnormal expression of homeobox genes and transthyretin in expansion carriers.

Neurol Genet 2017 Aug 7;3(4):e161. Epub 2017 Jun 7.

Department of Neuroscience (N.A.F., M.C.B., T.F.G., K.F.B., M.D.-H., P.H.B., J.C., K.R.J.-W., L.M.D., A.M.N., M.E.M., L.P., D.W.D., R.R., M.v.B.), Department of Health Sciences Research (X.W., Y.W.A.), Department of Neurology (N.R.G.-R., K.B.B.), Division of Biomedical Statistics and Informatics (M.G.H.), Mayo Clinic, Jacksonville, FL; Department of Neurology (J.W., M.B.), University of Miami, FL; Department of Neurology (K.A.J., J.E.P., D.S.K., R.C.P., B.F.B.), Mayo Clinic, Rochester, MN; and Divisions of Neurology and Neurobiology (R.B.), Barrow Neurological Institute, St. Joseph's Hospital and Medical Center, Phoenix, AZ.

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http://dx.doi.org/10.1212/NXG.0000000000000161DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5479438PMC
August 2017

A C9ORF72 BAC mouse model recapitulates key epigenetic perturbations of ALS/FTD.

Mol Neurodegener 2017 06 12;12(1):46. Epub 2017 Jun 12.

Department of Psychiatry & Behavioral Sciences, Center for Therapeutic Innovation, University of Miami Miller School of Medicine, 1501 NW 10th Ave, BRB-413, Miami, FL, 33136, USA.

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http://dx.doi.org/10.1186/s13024-017-0185-9DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5468954PMC
June 2017

Poly(GP) proteins are a useful pharmacodynamic marker for -associated amyotrophic lateral sclerosis.

Sci Transl Med 2017 03;9(383)

Department of Neuroscience, Mayo Clinic, Jacksonville, FL 32224, USA.

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http://dx.doi.org/10.1126/scitranslmed.aai7866DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5576451PMC
March 2017

Disease Mechanisms of Repeat Expansions.

Cold Spring Harb Perspect Med 2018 04 2;8(4). Epub 2018 Apr 2.

Department of Neuroscience, Mayo Clinic Florida, Jacksonville, Florida 32224.

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http://dx.doi.org/10.1101/cshperspect.a024224DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5880161PMC
April 2018

C9orf72 BAC Transgenic Mice Display Typical Pathologic Features of ALS/FTD.

Neuron 2015 Dec;88(5):892-901

Board of Governors Regenerative Medicine Institute, Cedars-Sinai Medical Center, 8700 Beverly Boulevard, Los Angeles, CA 90048, USA; Department of Neurology, Cedars-Sinai Medical Center, 8700 Beverly Boulevard, Los Angeles, CA 90048, USA. Electronic address:

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http://dx.doi.org/10.1016/j.neuron.2015.10.027DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4672384PMC
December 2015

TDP-1, the Caenorhabditis elegans ortholog of TDP-43, limits the accumulation of double-stranded RNA.

EMBO J 2014 Dec 12;33(24):2947-66. Epub 2014 Nov 12.

Institute for Behavioral Genetics University of Colorado, Boulder, CO, USA Integrative Physiology, University of Colorado, Boulder, CO, USA.

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http://dx.doi.org/10.15252/embj.201488740DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4282642PMC
December 2014

Characterization of DNA hypermethylation in the cerebellum of c9FTD/ALS patients.

Brain Res 2014 Oct 12;1584:15-21. Epub 2014 Feb 12.

Department of Research, Neuroscience, Mayo Clinic College of Medicine, 4500 San Pablo Road, Jacksonville, FL 32224, USA. Electronic address:

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http://dx.doi.org/10.1016/j.brainres.2014.02.015DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4130919PMC
October 2014

Mechanisms of toxicity in C9FTLD/ALS.

Acta Neuropathol 2014 Mar 7;127(3):359-76. Epub 2014 Jan 7.

Department of Neuroscience, Mayo Clinic Florida, Jacksonville, FL, 32224, USA.

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http://dx.doi.org/10.1007/s00401-013-1237-zDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4002260PMC
March 2014

Robust cytoplasmic accumulation of phosphorylated TDP-43 in transgenic models of tauopathy.

Acta Neuropathol 2013 Jul 11;126(1):39-50. Epub 2013 May 11.

Department of Neuroscience, Center for Translational Research in Neurodegenerative Disease, McKnight Brain Institute, University of Florida, 1275 Center Drive, BMS Building J-483, PO Box 100159, Gainesville, FL 32610-0244, USA.

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http://dx.doi.org/10.1007/s00401-013-1123-8DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3690181PMC
July 2013

Does obesity-induced τ phosphorylation tip the scale toward dementia?

Diabetes 2013 May;62(5):1365-6

Department of Neuroscience, Mayo Clinic Florida, Jacksonville, Florida, USA.

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http://dx.doi.org/10.2337/db12-1784DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3636650PMC
May 2013

RNA-mediated toxicity in neurodegenerative disease.

Mol Cell Neurosci 2013 Sep 29;56:406-19. Epub 2012 Dec 29.

Department of Neuroscience, Mayo Clinic, 4500 San Pablo Road, Jacksonville, FL 32224, USA.

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https://linkinghub.elsevier.com/retrieve/pii/S10447431120022
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http://dx.doi.org/10.1016/j.mcn.2012.12.006DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3791208PMC
September 2013

TARDBP mutation analysis in TDP-43 proteinopathies and deciphering the toxicity of mutant TDP-43.

J Alzheimers Dis 2013 ;33 Suppl 1:S35-45

Department of Neuroscience, Mayo Clinic, Jacksonville, FL 32224, USA.

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http://dx.doi.org/10.3233/JAD-2012-129036DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3532959PMC
June 2013

Loss of HDAC6, a novel CHIP substrate, alleviates abnormal tau accumulation.

Hum Mol Genet 2012 Jul 5;21(13):2936-45. Epub 2012 Apr 5.

Mayo Clinic, 4500 San Pablo Road, Jacksonville, FL 32224, USA.

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http://dx.doi.org/10.1093/hmg/dds125DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3373241PMC
July 2012

Rodent models of TDP-43 proteinopathy: investigating the mechanisms of TDP-43-mediated neurodegeneration.

J Mol Neurosci 2011 Nov 3;45(3):486-99. Epub 2011 Aug 3.

Department of Neuroscience, Mayo Clinic, 4500 San Pablo Road, Jacksonville, FL 32224, USA.

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http://link.springer.com/10.1007/s12031-011-9610-7
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http://dx.doi.org/10.1007/s12031-011-9610-7DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3207125PMC
November 2011

Phosphorylation regulates proteasomal-mediated degradation and solubility of TAR DNA binding protein-43 C-terminal fragments.

Mol Neurodegener 2010 Aug 30;5:33. Epub 2010 Aug 30.

Department of Neuroscience, Mayo Clinic, 4500 San Pablo Road, Jacksonville, Florida 32224, USA.

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http://dx.doi.org/10.1186/1750-1326-5-33DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2941488PMC
August 2010

Concentration-dependent effects of proteasomal inhibition on tau processing in a cellular model of tauopathy.

Int J Clin Exp Pathol 2009 Jun 15;2(6):561-73. Epub 2009 Jun 15.

Department of Neuroscience, Mayo Clinic College of Medicine Jacksonville, FL, USA.

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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2713451PMC
June 2009

The role of tau in neurodegeneration.

Mol Neurodegener 2009 Mar 11;4:13. Epub 2009 Mar 11.

Department of Neuroscience, Mayo Clinic College of Medicine, Jacksonville, Florida, USA.

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http://molecularneurodegeneration.biomedcentral.com/articles
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http://dx.doi.org/10.1186/1750-1326-4-13DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2663562PMC
March 2009

Ethanol enhances tau accumulation in neuroblastoma cells that inducibly express tau.

Neurosci Lett 2008 Oct 24;443(2):67-71. Epub 2008 Jul 24.

Department of Neuroscience, Mayo Clinic College of Medicine, Jacksonville, FL 32224, USA.

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http://dx.doi.org/10.1016/j.neulet.2008.07.052DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2570620PMC
October 2008

Autophagic-lysosomal perturbation enhances tau aggregation in transfectants with induced wild-type tau expression.

Eur J Neurosci 2008 Mar 22;27(5):1119-30. Epub 2008 Feb 22.

Department of Neuroscience, Mayo Clinic Jacksonville, Jacksonville, FL, USA.

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http://dx.doi.org/10.1111/j.1460-9568.2008.06084.xDOI Listing
March 2008

An alternative Ca2+-dependent mechanism of neuroprotection by the metalloporphyrin class of superoxide dismutase mimetics.

FASEB J 2005 Oct 4;19(12):1734-6. Epub 2005 Aug 4.

National Research Council, Institute for Biological Sciences, Synaptic Pathophysiology Group, Ottawa, ON, Canada.

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http://dx.doi.org/10.1096/fj.05-3795fjeDOI Listing
October 2005

The dual role of prostaglandin E(2) in excitotoxicity and preconditioning-induced neuroprotection.

Eur J Pharmacol 2005 Jul;517(1-2):17-27

University of Ottawa, Department of Cellular and Molecular Medicine, Ottawa, Ontario, Canada K1H 8M5.

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http://linkinghub.elsevier.com/retrieve/pii/S001429990500571
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http://dx.doi.org/10.1016/j.ejphar.2005.05.031DOI Listing
July 2005

Opposing effects of cyclooxygenase-2 selective inhibitors on oxygen-glucose deprivation-induced neurotoxicity.

Eur J Pharmacol 2004 Jun;493(1-3):45-55

Department of Cellular and Molecular Medicine, University of Ottawa, Ottawa, ON, Canada K1H 8M5.

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http://dx.doi.org/10.1016/j.ejphar.2004.04.026DOI Listing
June 2004