Retinoic acid worsens ATG10-dependent autophagy impairment in TBK1-mutant hiPSC-derived motoneurons through SQSTM1/p62 accumulation

Alberto Catanese(Universität Ulm), Florian olde Heuvel(Universität Ulm), Medhanie Mulaw, Maria Demestre(Universität Ulm), Julia Higelin(Universität Ulm), Gotthold Barbi, Axel Freischmidt(Universität Ulm), Jochen H. Weishaupt(Universität Ulm), Albert C. Ludolph(Universität Ulm), Francesco Roselli(Universität Ulm), Tobias M. Boeckers(Universität Ulm)
Autophagy
April 2, 2019
Cited by 61Open Access
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Abstract

4HPR: 4-hydroxy(phenyl)retinamide; AKT: AKT1 serine/threonine kinase 1; ALS: amyotrophic lateral sclerosis; ATG: autophagy related; AVs: autophagic vesicle; C9orf72: chromosome 9 open reading frame 72; CASP3: caspase 3; CHAT: choline O-acetyltransferase; CYCS: cytochrome c, somatic; DIV: day in vitro; FTD: frontotemporal dementia; FUS: FUS RNA binding protein; GFP: green fluorescent protein; hiPSCs: human induced pluripotent stem cells; MAP1LC3/LC3: microtubule associated protein 1 light chain 3; MNs: motoneurons; mRFP: monomeric red fluorescent protein; MTOR: mechanistic target of rapamycin kinase; NFE2L2/NRF2: nuclear factor, erythroid 2 like 2; RARA: retinoic acid receptor alpha; SLC18A3/VACHT: solute carrier family 18 (vesicular acetylcholine transporter), member 3; SQSTM1/p62: sequestosome 1; TBK1: TANK binding kinase 1; TEM: transmission electron microscopy.


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