271 related articles for article (PubMed ID: 32084385)
1. Knocking out C9ORF72 Exacerbates Axonal Trafficking Defects Associated with Hexanucleotide Repeat Expansion and Reduces Levels of Heat Shock Proteins.
Abo-Rady M; Kalmbach N; Pal A; Schludi C; Janosch A; Richter T; Freitag P; Bickle M; Kahlert AK; Petri S; Stefanov S; Glass H; Staege S; Just W; Bhatnagar R; Edbauer D; Hermann A; Wegner F; Sterneckert JL
Stem Cell Reports; 2020 Mar; 14(3):390-405. PubMed ID: 32084385
[TBL] [Abstract][Full Text] [Related]
2. Correction of amyotrophic lateral sclerosis related phenotypes in induced pluripotent stem cell-derived motor neurons carrying a hexanucleotide expansion mutation in C9orf72 by CRISPR/Cas9 genome editing using homology-directed repair.
Ababneh NA; Scaber J; Flynn R; Douglas A; Barbagallo P; Candalija A; Turner MR; Sims D; Dafinca R; Cowley SA; Talbot K
Hum Mol Genet; 2020 Aug; 29(13):2200-2217. PubMed ID: 32504093
[TBL] [Abstract][Full Text] [Related]
3. C9orf72 Hexanucleotide Expansions Are Associated with Altered Endoplasmic Reticulum Calcium Homeostasis and Stress Granule Formation in Induced Pluripotent Stem Cell-Derived Neurons from Patients with Amyotrophic Lateral Sclerosis and Frontotemporal Dementia.
Dafinca R; Scaber J; Ababneh N; Lalic T; Weir G; Christian H; Vowles J; Douglas AG; Fletcher-Jones A; Browne C; Nakanishi M; Turner MR; Wade-Martins R; Cowley SA; Talbot K
Stem Cells; 2016 Aug; 34(8):2063-78. PubMed ID: 27097283
[TBL] [Abstract][Full Text] [Related]
4. Mitochondrial bioenergetic deficits in C9orf72 amyotrophic lateral sclerosis motor neurons cause dysfunctional axonal homeostasis.
Mehta AR; Gregory JM; Dando O; Carter RN; Burr K; Nanda J; Story D; McDade K; Smith C; Morton NM; Mahad DJ; Hardingham GE; Chandran S; Selvaraj BT
Acta Neuropathol; 2021 Feb; 141(2):257-279. PubMed ID: 33398403
[TBL] [Abstract][Full Text] [Related]
5. Concomitant gain and loss of function pathomechanisms in C9ORF72 amyotrophic lateral sclerosis.
Pal A; Kretner B; Abo-Rady M; Glaβ H; Dash BP; Naumann M; Japtok J; Kreiter N; Dhingra A; Heutink P; Böckers TM; Günther R; Sterneckert J; Hermann A
Life Sci Alliance; 2021 Apr; 4(4):. PubMed ID: 33619157
[TBL] [Abstract][Full Text] [Related]
6. Targeting RNA foci in iPSC-derived motor neurons from ALS patients with a C9ORF72 repeat expansion.
Sareen D; O'Rourke JG; Meera P; Muhammad AK; Grant S; Simpkinson M; Bell S; Carmona S; Ornelas L; Sahabian A; Gendron T; Petrucelli L; Baughn M; Ravits J; Harms MB; Rigo F; Bennett CF; Otis TS; Svendsen CN; Baloh RH
Sci Transl Med; 2013 Oct; 5(208):208ra149. PubMed ID: 24154603
[TBL] [Abstract][Full Text] [Related]
7. Expression of C9orf72 hexanucleotide repeat expansion leads to formation of RNA foci and dipeptide repeat proteins but does not influence autophagy or proteasomal function in neuronal cells.
Leskelä S; Huber N; Hoffmann D; Rostalski H; Remes AM; Takalo M; Hiltunen M; Haapasalo A
Biochim Biophys Acta Mol Cell Res; 2021 Jun; 1868(7):119021. PubMed ID: 33775797
[TBL] [Abstract][Full Text] [Related]
8. A C9ORF72 BAC mouse model recapitulates key epigenetic perturbations of ALS/FTD.
Esanov R; Cabrera GT; Andrade NS; Gendron TF; Brown RH; Benatar M; Wahlestedt C; Mueller C; Zeier Z
Mol Neurodegener; 2017 Jun; 12(1):46. PubMed ID: 28606110
[TBL] [Abstract][Full Text] [Related]
9. UBQLN2-HSP70 axis reduces poly-Gly-Ala aggregates and alleviates behavioral defects in the C9ORF72 animal model.
Zhang K; Wang A; Zhong K; Qi S; Wei C; Shu X; Tu WY; Xu W; Xia C; Xiao Y; Chen A; Bai L; Zhang J; Luo B; Wang W; Shen C
Neuron; 2021 Jun; 109(12):1949-1962.e6. PubMed ID: 33991504
[TBL] [Abstract][Full Text] [Related]
10. ADAR2 mislocalization and widespread RNA editing aberrations in C9orf72-mediated ALS/FTD.
Moore S; Alsop E; Lorenzini I; Starr A; Rabichow BE; Mendez E; Levy JL; Burciu C; Reiman R; Chew J; Belzil VV; W Dickson D; Robertson J; Staats KA; Ichida JK; Petrucelli L; Van Keuren-Jensen K; Sattler R
Acta Neuropathol; 2019 Jul; 138(1):49-65. PubMed ID: 30945056
[TBL] [Abstract][Full Text] [Related]
11. C9orf72 and RAB7L1 regulate vesicle trafficking in amyotrophic lateral sclerosis and frontotemporal dementia.
Aoki Y; Manzano R; Lee Y; Dafinca R; Aoki M; Douglas AGL; Varela MA; Sathyaprakash C; Scaber J; Barbagallo P; Vader P; Mäger I; Ezzat K; Turner MR; Ito N; Gasco S; Ohbayashi N; El Andaloussi S; Takeda S; Fukuda M; Talbot K; Wood MJA
Brain; 2017 Apr; 140(4):887-897. PubMed ID: 28334866
[TBL] [Abstract][Full Text] [Related]
12. Ablation of C9orf72 together with excitotoxicity induces ALS in rats.
Dong W; Ma Y; Guan F; Zhang X; Chen W; Zhang L; Zhang L
FEBS J; 2021 Mar; 288(5):1712-1723. PubMed ID: 32745320
[TBL] [Abstract][Full Text] [Related]
13. Haploinsufficiency leads to neurodegeneration in C9ORF72 ALS/FTD human induced motor neurons.
Shi Y; Lin S; Staats KA; Li Y; Chang WH; Hung ST; Hendricks E; Linares GR; Wang Y; Son EY; Wen X; Kisler K; Wilkinson B; Menendez L; Sugawara T; Woolwine P; Huang M; Cowan MJ; Ge B; Koutsodendris N; Sandor KP; Komberg J; Vangoor VR; Senthilkumar K; Hennes V; Seah C; Nelson AR; Cheng TY; Lee SJ; August PR; Chen JA; Wisniewski N; Hanson-Smith V; Belgard TG; Zhang A; Coba M; Grunseich C; Ward ME; van den Berg LH; Pasterkamp RJ; Trotti D; Zlokovic BV; Ichida JK
Nat Med; 2018 Mar; 24(3):313-325. PubMed ID: 29400714
[TBL] [Abstract][Full Text] [Related]
14. Stable transgenic C9orf72 zebrafish model key aspects of the ALS/FTD phenotype and reveal novel pathological features.
Shaw MP; Higginbottom A; McGown A; Castelli LM; James E; Hautbergue GM; Shaw PJ; Ramesh TM
Acta Neuropathol Commun; 2018 Nov; 6(1):125. PubMed ID: 30454072
[TBL] [Abstract][Full Text] [Related]
15. A toxic gain-of-function mechanism in C9orf72 ALS impairs the autophagy-lysosome pathway in neurons.
Beckers J; Tharkeshwar AK; Fumagalli L; Contardo M; Van Schoor E; Fazal R; Thal DR; Chandran S; Mancuso R; Van Den Bosch L; Van Damme P
Acta Neuropathol Commun; 2023 Sep; 11(1):151. PubMed ID: 37723585
[TBL] [Abstract][Full Text] [Related]
16. UPF1 reduces C9orf72 HRE-induced neurotoxicity in the absence of nonsense-mediated decay dysfunction.
Zaepfel BL; Zhang Z; Maulding K; Coyne AN; Cheng W; Hayes LR; Lloyd TE; Sun S; Rothstein JD
Cell Rep; 2021 Mar; 34(13):108925. PubMed ID: 33789100
[TBL] [Abstract][Full Text] [Related]
17. The DNA damage response (DDR) is induced by the C9orf72 repeat expansion in amyotrophic lateral sclerosis.
Farg MA; Konopka A; Soo KY; Ito D; Atkin JD
Hum Mol Genet; 2017 Aug; 26(15):2882-2896. PubMed ID: 28481984
[TBL] [Abstract][Full Text] [Related]
18. Mutant C9orf72 human iPSC-derived astrocytes cause non-cell autonomous motor neuron pathophysiology.
Zhao C; Devlin AC; Chouhan AK; Selvaraj BT; Stavrou M; Burr K; Brivio V; He X; Mehta AR; Story D; Shaw CE; Dando O; Hardingham GE; Miles GB; Chandran S
Glia; 2020 May; 68(5):1046-1064. PubMed ID: 31841614
[TBL] [Abstract][Full Text] [Related]
19. Dipeptide repeat proteins activate a heat shock response found in C9ORF72-ALS/FTLD patients.
Mordes DA; Prudencio M; Goodman LD; Klim JR; Moccia R; Limone F; Pietilainen O; Chowdhary K; Dickson DW; Rademakers R; Bonini NM; Petrucelli L; Eggan K
Acta Neuropathol Commun; 2018 Jul; 6(1):55. PubMed ID: 29973287
[TBL] [Abstract][Full Text] [Related]
20. Altered calcium dynamics and glutamate receptor properties in iPSC-derived motor neurons from ALS patients with C9orf72, FUS, SOD1 or TDP43 mutations.
Bursch F; Kalmbach N; Naujock M; Staege S; Eggenschwiler R; Abo-Rady M; Japtok J; Guo W; Hensel N; Reinhardt P; Boeckers TM; Cantz T; Sterneckert J; Van Den Bosch L; Hermann A; Petri S; Wegner F
Hum Mol Genet; 2019 Sep; 28(17):2835-2850. PubMed ID: 31108504
[TBL] [Abstract][Full Text] [Related]
[Next] [New Search]
