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TitleIn Situ Structure of Neuronal C9orf72 Poly-GA Aggregates Reveals Proteasome Recruitment.
Journal, issue, pagesCell, Vol. 172, Issue 4, Page 696-705.e12, Year 2018
Publish dateFeb 8, 2018
AuthorsQiang Guo / Carina Lehmer / Antonio Martínez-Sánchez / Till Rudack / Florian Beck / Hannelore Hartmann / Manuela Pérez-Berlanga / Frédéric Frottin / Mark S Hipp / F Ulrich Hartl / Dieter Edbauer / Wolfgang Baumeister / Rubén Fernández-Busnadiego /
PubMed AbstractProtein 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- ...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 at high resolution. We focus on the poly-Gly-Ala (poly-GA) aggregates resulting from aberrant translation of an expanded GGGGCC repeat in C9orf72, the most common genetic cause of amyotrophic lateral sclerosis and frontotemporal dementia. We find that poly-GA aggregates consist of densely packed twisted ribbons that recruit numerous 26S proteasome complexes, while other macromolecules are largely excluded. Proximity to poly-GA ribbons stabilizes a transient substrate-processing conformation of the 26S proteasome, suggesting stalled degradation. Thus, poly-GA aggregates may compromise neuronal proteostasis by driving the accumulation and functional impairment of a large fraction of cellular proteasomes.
External linksCell / PubMed:29398115 / PubMed Central
MethodsEM (subtomogram averaging) / EM (tomography)
Resolution11.8 - 17.1 Å
Structure data

3913

6epc

EMDB-3913, PDB-6epc:
Ground state 26S proteasome (GS2)
Method: EM (subtomogram averaging) / Resolution: 12.3 Å

3914

6epd

EMDB-3914, PDB-6epd:
Substrate processing state 26S proteasome (SPS1)
Method: EM (subtomogram averaging) / Resolution: 15.4 Å

3915

6epe

EMDB-3915, PDB-6epe:
Substrate processing state 26S proteasome (SPS2)
Method: EM (subtomogram averaging) / Resolution: 12.8 Å

3916

6epf

EMDB-3916, PDB-6epf:
Ground state 26S proteasome (GS1)
Method: EM (subtomogram averaging) / Resolution: 11.8 Å

EMDB-3917:
Rat TRiC structure
Method: EM (subtomogram averaging) / Resolution: 17.1 Å

EMDB-4191:
In situ cryo-electron tomogram from Rat neuron with C9ORF72 Poly-GA aggregates
Method: EM (tomography)

Source
  • rattus norvegicus (Norway rat)
  • Rat (rat)
  • Norway Rat (Norway rat)
KeywordsHYDROLASE / UPS / Ground state / Neuron degeneration / Substrate processing state

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