Nuclear egress of TDP-43 and FUS occurs independently of Exportin-1/CRM1

Autor: Helena Ederle, Christina Funk, Claudia Abou-Ajram, Saskia Hutten, Eva B. E. Funk, Ralph H. Kehlenbach, Susanne M. Bailer, Dorothee Dormann

Publikováno v: Scientific Reports, Vol 8, Iss 1, Pp 1-18 (2018)

Abstract TDP-43 and FUS are nuclear proteins with multiple functions in mRNA processing. They play key roles in ALS (amyotrophic lateral sclerosis) and FTD (frontotemporal dementia), where they are partially lost from the nucleus and aggregate in the

Externí odkaz: https://doaj.org/article/8094611fcf1f43b9ba32a1e651adbced

Cytoplasmic poly-GA aggregates impair nuclear import of TDP-43 in C9orf72 ALS/FTLD

Autor: Dieter Edbauer, Bahram Khosravi, Dorothee Dormann, Helena Ederle, Christoph Möhl, Martin H. Schludi, Meike Michaelsen, Hannelore Hartmann, Stephanie May

Publikováno v: Human Molecular Genetics
Human molecular genetics 26(4), ddw432 (2016). doi:10.1093/hmg/ddw432

A repeat expansion in the non-coding region of C9orf72 gene is the most common mutation causing frontotemporal lobar degeneration (FTLD) and amyotrophic lateral sclerosis (ALS). Sense and antisense transcripts are translated into aggregating dipeptid

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::1e42f8f3c60e67b5398c850f2e78d39a
http://europepmc.org/articles/PMC5409121

Hypertonic Stress Causes Cytoplasmic Translocation of Neuronal, but Not Astrocytic, FUS due to Impaired Transportin Function

Autor: Dorothee Dormann, Stefan Reber, Luc Dupuis, Helena Ederle, Marian Hruska-Plochan, Eva-Maria Hock, Marc-David Ruepp, Florent Laferrière, Zuzanna Maniecka, Lucas Pelkmans, M K Sonu Sahadevan, Tammaryn Lashley, Lauren M. Gittings, Magdalini Polymenidou

Publikováno v: Cell Reports
Cell Reports, 2018, 24 (4), pp.987-1000.e7. ⟨10.1016/j.celrep.2018.06.094⟩
Cell Reports, Elsevier Inc, 2018, 24 (4), pp.987-1000.e7. ⟨10.1016/j.celrep.2018.06.094⟩
Hock, E-M, Maniecka, Z, Hruska-Plochan, M, Reber, S, Laferrière, F, Sahadevan M K, S, Ederle, H, Gittings, L, Pelkmans, L, Dupuis, L, Lashley, T, Ruepp, M-D, Dormann, D & Polymenidou, M 2018, ' Hypertonic Stress Causes Cytoplasmic Translocation of Neuronal, but Not Astrocytic, FUS due to Impaired Transportin Function ', Cell Reports, vol. 24, no. 4, pp. 987-1000.e7 . https://doi.org/10.1016/j.celrep.2018.06.094
Cell Reports, 24 (4)

The primarily nuclear RNA-binding protein FUS (fused in sarcoma) forms pathological cytoplasmic inclusions in a subset of early-onset amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD) patients. In response to cellular stress, FUS

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::d26cd76ee2b60a0880af6400794f15f6
https://doi.org/10.1016/j.celrep.2018.06.094