Cited by 631Autophagy in Disease and TherapyCellular transport and secretionAmyotrophic Lateral Sclerosis Research

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Functional multivesicular bodies are required for autophagic clearance of protein aggregates associated with neurodegenerative disease

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Filimonenko, Maria, Stuffers, Susanne, Raiborg, Camilla, Yamamoto, Ai, Malerød, Lene, Fisher, Elizabeth M.C., Isaacs, Adrian, Brech, Andreas, Stenmark, Harald, Simonsen, Anne·2007·The Journal of Cell Biology

DOI 10.1083/jcb.200702115PMC PMC2064794

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Abstract

The endosomal sorting complexes required for transport (ESCRTs) are required to sort integral membrane proteins into intralumenal vesicles of the multivesicular body (MVB). Mutations in the ESCRT-III subunit CHMP2B were recently associated with frontotemporal dementia and amyotrophic lateral sclerosis (ALS), neurodegenerative diseases characterized by abnormal ubiquitin-positive protein deposits in affected neurons. We show here that autophagic degradation is inhibited in cells depleted of ESCRT subunits and in cells expressing CHMP2B mutants, leading to accumulation of protein aggregates containing ubiquitinated proteins, p62 and Alfy. Moreover, we find that functional MVBs are required for clearance of TDP-43 (identified as the major ubiquitinated protein in ALS and frontotemporal lobar degeneration with ubiquitin deposits), and of expanded polyglutamine aggregates associated with Huntington's disease. Together, our data indicate that efficient autophagic degradation requires functional MVBs and provide a possible explanation to the observed neurodegenerative phenotype seen in patients with CHMP2B mutations.

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Authors: Filimonenko, M., Stuffers, S., Raiborg, C., Yamamoto, A., Malerød, L., Fisher, E. M. C., Isaacs, A., Brech, A., Stenmark, H., & Simonsen, A.
Affiliations: University of Oslo (NO)
Columbia University (US)
University College London (GB)
MRC Prion Unit (GB)
Corresponding author: Simonsen, Anne
ORCID: Raiborg, Camilla
Yamamoto, Ai
Malerød, Lene
Fisher, Elizabeth M.C.
Isaacs, Adrian
Brech, Andreas
Stenmark, Harald
Simonsen, Anne
Venue: The Journal of Cell Biology
Publisher: Rockefeller University Press
Published: 2007-11-05
Volume: 179
Issue: 3
Pages: 485-500
ISSN: 0021-9525 (ISSN-L)
1540-8140
DOI: 10.1083/jcb.200702115
PMC: PMC2064794
OpenAlex: W2103275390
Cited by: 626
References: 62
Citations by year: 2026: 13 · 2025: 26 · 2024: 20 · 2023: 15 · 2022: 18 · 2021: 35 · 2020: 29 · 2019: 27 · 2018: 41 · 2017: 31 · 2016: 46 · 2015: 34 · 2014: 43 · 2013: 36 · 2012: 40
FWCI: 29.80
Type: journal article
Language: en
Open access: bronze
doi.org
Locations: The Journal of Cell Biology (publishedVersion)
PubMed Central (publishedVersion, cc-by-nc-sa)
PubMed (publishedVersion)
UCL Discovery (University College London) (submittedVersion)
Topics: Life Sciences › Biochemistry, Genetics and Molecular Biology › Cell Biology › Autophagy in Disease and Therapy
Life Sciences › Biochemistry, Genetics and Molecular Biology › Cell Biology › Cellular transport and secretion
Health Sciences › Medicine › Clinical Neurology › Amyotrophic Lateral Sclerosis Research
Keywords: multivesicular bodies, ESCRT, CHMP2B, autophagy, protein aggregates, neurodegenerative diseases, TDP-43, polyglutamine aggregates, Huntington's disease, amyotrophic lateral sclerosis, frontotemporal dementia, p62, ESCRT-III
MeSH terms: Autophagy, Cell Membrane, HeLa Cells, Humans, Huntington Disease, Models, Biological, Mutation, Nerve Tissue Proteins, Neurons, Phenotype, Protein Binding, Endosomes, Neurodegenerative Diseases, Ubiquitin, Endosomal Sorting Complexes Required for Transport

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Functional multivesicular bodies are required for autophagic clearance of protein aggregates associated with neurodegenerative disease

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