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The ALS-linked E102Q mutation in Sigma receptor-1 leads to ER stress-mediated defects in protein homeostasis and dysregulation of RNA-binding proteins.
Dreser A, Vollrath JT, Sechi A, Johann S, Roos A, Yamoah A, Katona I, Bohlega S, Wiemuth D, Tian Y, Schmidt A, Vervoorts J, Dohmen M, Beyer C, Anink J, Aronica E, Troost D, Weis J, Goswami A. Dreser A, et al. Among authors: troost d. Cell Death Differ. 2017 Oct;24(10):1655-1671. doi: 10.1038/cdd.2017.88. Epub 2017 Jun 16. Cell Death Differ. 2017. PMID: 28622300 Free PMC article.
ALS-Associated Endoplasmic Reticulum Proteins in Denervated Skeletal Muscle: Implications for Motor Neuron Disease Pathology.
Jesse CM, Bushuven E, Tripathi P, Chandrasekar A, Simon CM, Drepper C, Yamoah A, Dreser A, Katona I, Johann S, Beyer C, Wagner S, Grond M, Nikolin S, Anink J, Troost D, Sendtner M, Goswami A, Weis J. Jesse CM, et al. Among authors: troost d. Brain Pathol. 2017 Nov;27(6):781-794. doi: 10.1111/bpa.12453. Epub 2017 Feb 15. Brain Pathol. 2017. PMID: 27790792 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: troost d. Nat Commun. 2018 Jan 23;9(1):335. doi: 10.1038/s41467-017-02299-1. Nat Commun. 2018. PMID: 29362359 Free PMC article.
FUS pathology in ALS is linked to alterations in multiple ALS-associated proteins and rescued by drugs stimulating autophagy.
Marrone L, Drexler HCA, Wang J, Tripathi P, Distler T, Heisterkamp P, Anderson EN, Kour S, Moraiti A, Maharana S, Bhatnagar R, Belgard TG, Tripathy V, Kalmbach N, Hosseinzadeh Z, Crippa V, Abo-Rady M, Wegner F, Poletti A, Troost D, Aronica E, Busskamp V, Weis J, Pandey UB, Hyman AA, Alberti S, Goswami A, Sterneckert J. Marrone L, et al. Among authors: troost d. Acta Neuropathol. 2019 Jul;138(1):67-84. doi: 10.1007/s00401-019-01998-x. Epub 2019 Apr 1. Acta Neuropathol. 2019. PMID: 30937520 Free PMC article.
213 results