In Situ Structure of Neuronal C9orf72 Poly-GA Aggregates Reveals Proteasome Recruitment

Protein aggregation and dysfunction of the ubiquitin-proteasome system are hallmarks of many neurodegenerative diseases. Here, we address the elusive link between these phenomena by employing cryo-electron tomography to dissect the molecular architecture of protein aggregates within intact neurons a...

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Veröffentlicht in:	Cell 2018-02, Vol.172 (4), p.696-705.e12
Hauptverfasser:	Guo, Qiang, Lehmer, Carina, Martínez-Sánchez, Antonio, Rudack, Till, Beck, Florian, Hartmann, Hannelore, Pérez-Berlanga, Manuela, Frottin, Frédéric, Hipp, Mark S., Hartl, F. Ulrich, Edbauer, Dieter, Baumeister, Wolfgang, Fernández-Busnadiego, Rubén
Format:	Artikel
Sprache:	eng
Schlagworte:	Alanine - analogs & derivatives Alanine - genetics Alanine - metabolism ALS amyotrophic lateral sclerosis Amyotrophic Lateral Sclerosis - genetics Amyotrophic Lateral Sclerosis - metabolism Amyotrophic Lateral Sclerosis - pathology Animals C9orf72 Protein - genetics C9orf72 Protein - metabolism cryo-EM cryo-ET cryo-FIB cryoelectron microscopy dementia dipeptide-repeat proteins Frontotemporal Dementia - genetics Frontotemporal Dementia - metabolism Frontotemporal Dementia - pathology FTD HEK293 Cells Humans neurons Neurons - metabolism Neurons - pathology Polyglutamic Acid - genetics Polyglutamic Acid - metabolism proteasome endopeptidase complex Proteasome Endopeptidase Complex - genetics Proteasome Endopeptidase Complex - metabolism Protein Aggregates Protein Biosynthesis Protein Stability Protein Structure, Quaternary Rats Rats, Sprague-Dawley tomography translation (genetics) UPS
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