Wild type human TDP-43 potentiates ALS-linked mutant TDP-43 driven progressive motor and cortical neuron degeneration with pathological features of ALS

被引:0
|
作者
Jacqueline C Mitchell
Remy Constable
Eva So
Caroline Vance
Emma Scotter
Leanne Glover
Tibor Hortobagyi
Eveline S. Arnold
Shuo-Chien Ling
Melissa McAlonis
Sandrine Da Cruz
Magda Polymenidou
Lino Tessarolo
Don W Cleveland
Christopher E Shaw
机构
[1] Institute of Psychiatry,King’s Centre for Neurodegeneration Research, Kings College London, Department of Basic and Clinical Neurosciences
[2] Psychology and Neuroscience,Ludwig Institute for Cancer Research and Department of Molecular Medicine
[3] Centre for Ultrastructural Imaging,undefined
[4] Kings College London,undefined
[5] University of California at San Diego,undefined
[6] Mouse Cancer Genetics Program,undefined
[7] National Cancer Institute – Frederick,undefined
[8] Present address: Neurogenetics Branch,undefined
[9] NINDS,undefined
[10] National Institutes of Health,undefined
[11] Present address: Institute of Molecular Life Sciences,undefined
[12] University of Zurich,undefined
关键词
TDP-43; Amyotrophic lateral sclerosis; Mouse;
D O I
暂无
中图分类号
学科分类号
摘要
引用
收藏
相关论文
共 50 条
  • [41] The ALS disease protein TDP-43 is actively transported in motor neuron axons and regulates axon outgrowth
    Fallini, Claudia
    Bassell, Gary J.
    Rossoll, Wilfried
    HUMAN MOLECULAR GENETICS, 2012, 21 (16) : 3703 - 3718
  • [42] Senataxin mutations elicit motor neuron degeneration phenotypes and yield TDP-43 mislocalization in ALS4 mice and human patients
    Bennett, Craig L.
    Dastidar, Somasish G.
    Ling, Shuo-Chien
    Malik, Bilal
    Ashe, Travis
    Wadhwa, Mandheer
    Miller, Derek B.
    Lee, Changwoo
    Mitchell, Matthew B.
    van Es, Michael A.
    Grunseich, Christopher
    Chen, Yingzhang
    Sopher, Bryce L.
    Greensmith, Linda
    Cleveland, Don W.
    La Spada, Albert R.
    ACTA NEUROPATHOLOGICA, 2018, 136 (03) : 425 - 443
  • [43] Mutant TDP-43 Causes Early-Stage Dose-Dependent Motor Neuron Degeneration in a TARDBP Knockin Mouse Model of ALS
    Ebstein, Sarah Y.
    Yagudayeva, Ilona
    Shneider, Neil A.
    CELL REPORTS, 2019, 26 (02): : 364 - +
  • [44] Senataxin mutations elicit motor neuron degeneration phenotypes and yield TDP-43 mislocalization in ALS4 mice and human patients
    Craig L. Bennett
    Somasish G. Dastidar
    Shuo-Chien Ling
    Bilal Malik
    Travis Ashe
    Mandheer Wadhwa
    Derek B. Miller
    Changwoo Lee
    Matthew B. Mitchell
    Michael A. van Es
    Christopher Grunseich
    Yingzhang Chen
    Bryce L. Sopher
    Linda Greensmith
    Don W. Cleveland
    Albert R. La Spada
    Acta Neuropathologica, 2018, 136 : 425 - 443
  • [45] Amygdala TDP-43 Pathology in Frontotemporal Lobar Degeneration and Motor Neuron Disease
    Takeda, Takahiro
    Seilhean, Danielle
    Le Ber, Isabelle
    Millecamps, Stephanie
    Sazdovitch, Veronique
    Kitagawa, Kazuo
    Uchihara, Toshiki
    Duyckaerts, Charles
    JOURNAL OF NEUROPATHOLOGY AND EXPERIMENTAL NEUROLOGY, 2017, 76 (09): : 800 - 812
  • [46] The ALS disease-associated mutant TDP-43 impairs mitochondrial dynamics and function in motor neurons
    Wang, Wenzhang
    Li, Li
    Lin, Wen-Lang
    Dickson, Dennis W.
    Petrucelli, Leonard
    Zhang, Teng
    Wang, Xinglong
    HUMAN MOLECULAR GENETICS, 2013, 22 (23) : 4706 - 4719
  • [47] ALS-linked TDP-43 mutations interfere with the recruitment of RNA recognition motifs to G-quadruplex RNA
    Ishiguro, Akira
    Ishihama, Akira
    SCIENTIFIC REPORTS, 2023, 13 (01)
  • [48] TMEM106B modifies TDP-43 pathology in human ALS brain and cell-based models of TDP-43 proteinopathy
    Mao, Fei
    Robinson, John L.
    Unger, Travis
    Posavi, Marijan
    Amado, Defne A.
    Elman, Lauren
    Grossman, Murray
    Wolk, David A.
    Lee, Edward B.
    Van Deerlin, Vivianna M.
    Porta, Silvia
    Lee, Virginia M. Y.
    Trojanowski, John Q.
    Chen-Plotkin, Alice S.
    ACTA NEUROPATHOLOGICA, 2021, 142 (04) : 629 - 642
  • [49] Essential Roles and Risks of G-Quadruplex Regulation: Recognition Targets of ALS-Linked TDP-43 and FUS
    Ishiguro, Akira
    Ishihama, Akira
    FRONTIERS IN MOLECULAR BIOSCIENCES, 2022, 9
  • [50] Wild-type and A315T mutant TDP-43 exert differential neurotoxicity in a Drosophila model of ALS
    Estes, Patricia S.
    Boehringer, Ashley
    Zwick, Rebecca
    Tang, Jonathan E.
    Grigsby, Brianna
    Zarnescu, Daniela C.
    HUMAN MOLECULAR GENETICS, 2011, 20 (12) : 2308 - 2321