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69 results

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Human iPSC models of neuronal ceroid lipofuscinosis capture distinct effects of TPP1 and CLN3 mutations on the endocytic pathway.
Lojewski X, Staropoli JF, Biswas-Legrand S, Simas AM, Haliw L, Selig MK, Coppel SH, Goss KA, Petcherski A, Chandrachud U, Sheridan SD, Lucente D, Sims KB, Gusella JF, Sondhi D, Crystal RG, Reinhardt P, Sterneckert J, Schöler H, Haggarty SJ, Storch A, Hermann A, Cotman SL. Lojewski X, et al. Among authors: sterneckert j. Hum Mol Genet. 2014 Apr 15;23(8):2005-22. doi: 10.1093/hmg/ddt596. Epub 2013 Nov 23. Hum Mol Genet. 2014. PMID: 24271013 Free PMC article.
4-Aminopyridine Induced Activity Rescues Hypoexcitable Motor Neurons from Amyotrophic Lateral Sclerosis Patient-Derived Induced Pluripotent Stem Cells.
Naujock M, Stanslowsky N, Bufler S, Naumann M, Reinhardt P, Sterneckert J, Kefalakes E, Kassebaum C, Bursch F, Lojewski X, Storch A, Frickenhaus M, Boeckers TM, Putz S, Demestre M, Liebau S, Klingenstein M, Ludolph AC, Dengler R, Kim KS, Hermann A, Wegner F, Petri S. Naujock M, et al. Among authors: sterneckert j. Stem Cells. 2016 Jun;34(6):1563-75. doi: 10.1002/stem.2354. Epub 2016 Mar 28. Stem Cells. 2016. PMID: 26946488
C9ORF72 interaction with cofilin modulates actin dynamics in motor neurons.
Sivadasan R, Hornburg D, Drepper C, Frank N, Jablonka S, Hansel A, Lojewski X, Sterneckert J, Hermann A, Shaw PJ, Ince PG, Mann M, Meissner F, Sendtner M. Sivadasan R, et al. Among authors: sterneckert j. Nat Neurosci. 2016 Dec;19(12):1610-1618. doi: 10.1038/nn.4407. Epub 2016 Oct 10. Nat Neurosci. 2016. PMID: 27723745 Free article.
Neuronal Dysfunction in iPSC-Derived Medium Spiny Neurons from Chorea-Acanthocytosis Patients Is Reversed by Src Kinase Inhibition and F-Actin Stabilization.
Stanslowsky N, Reinhardt P, Glass H, Kalmbach N, Naujock M, Hensel N, Lübben V, Pal A, Venneri A, Lupo F, De Franceschi L, Claus P, Sterneckert J, Storch A, Hermann A, Wegner F. Stanslowsky N, et al. Among authors: sterneckert j. J Neurosci. 2016 Nov 23;36(47):12027-12043. doi: 10.1523/JNEUROSCI.0456-16.2016. J Neurosci. 2016. PMID: 27881786 Free PMC article.
HDAC6 inhibition reverses axonal transport defects in motor neurons derived from FUS-ALS patients.
Guo W, Naujock M, Fumagalli L, Vandoorne T, Baatsen P, Boon R, Ordovás L, Patel A, Welters M, Vanwelden T, Geens N, Tricot T, Benoy V, Steyaert J, Lefebvre-Omar C, Boesmans W, Jarpe M, Sterneckert J, Wegner F, Petri S, Bohl D, Vanden Berghe P, Robberecht W, Van Damme P, Verfaillie C, Van Den Bosch L. Guo W, et al. Among authors: sterneckert j. Nat Commun. 2017 Oct 11;8(1):861. doi: 10.1038/s41467-017-00911-y. Nat Commun. 2017. PMID: 29021520 Free PMC article.
Isogenic FUS-eGFP iPSC Reporter Lines Enable Quantification of FUS Stress Granule Pathology that Is Rescued by Drugs Inducing Autophagy.
Marrone L, Poser I, Casci I, Japtok J, Reinhardt P, Janosch A, Andree C, Lee HO, Moebius C, Koerner E, Reinhardt L, Cicardi ME, Hackmann K, Klink B, Poletti A, Alberti S, Bickle M, Hermann A, Pandey UB, Hyman AA, Sterneckert JL. Marrone L, et al. Among authors: sterneckert jl. Stem Cell Reports. 2018 Feb 13;10(2):375-389. doi: 10.1016/j.stemcr.2017.12.018. Epub 2018 Jan 18. Stem Cell Reports. 2018. PMID: 29358088 Free PMC article.
Impaired DNA damage response signaling by FUS-NLS mutations leads to neurodegeneration and FUS aggregate formation.
Naumann M, Pal A, Goswami A, Lojewski X, Japtok J, Vehlow A, Naujock M, Günther R, Jin M, Stanslowsky N, Reinhardt P, Sterneckert J, Frickenhaus M, Pan-Montojo F, Storkebaum E, Poser I, Freischmidt A, Weishaupt JH, Holzmann K, Troost D, Ludolph AC, Boeckers TM, Liebau S, Petri S, Cordes N, Hyman AA, Wegner F, Grill SW, Weis J, Storch A, Hermann A. Naumann M, et al. Among authors: sterneckert j. Nat Commun. 2018 Jan 23;9(1):335. doi: 10.1038/s41467-017-02299-1. Nat Commun. 2018. PMID: 29362359 Free PMC article.
69 results