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

Functional multivesicular bodies are required for autophagic clearance of protein aggregates associated with neurodegenerative disease. Research Abstract Details 

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Functional multivesicular bodies are required for autophagic clearance of protein aggregates associated with neurodegenerative disease. Abstract Text:

Maria Filimonenko,Susanne Stuffers,Camilla Raiborg,Ai Yamamoto,Lene ,Elizabeth M C Fisher,Adrian Isaacs,Andreas Brech,Harald Stenmark,Anne Simonsen,Maria Filimonenko,Susanne Stuffers,Camilla Raiborg,Ai Yamamoto,Lene ,Elizabeth M C Fisher,Adrian Isaacs,Andreas Brech,Harald Stenmark,Anne Simonsen,

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.

Functional multivesicular bodies are required for autophagic clearance of protein aggregates associated with neurodegenerative disease. Publishing Authors By Initials

M Filimonenko,S Stuffers,C Raiborg,A Yamamoto,L ,EM Fisher,A Isaacs,A Brech,H Stenmark,A Simonsen,M Filimonenko,S Stuffers,C Raiborg,A Yamamoto,L ,EM Fisher,A Isaacs,A Brech,H Stenmark,A Simonsen,

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Functional multivesicular bodies are required for autophagic clearance of protein aggregates associated with neurodegenerative disease. Journal Published:

PUBLICATION TYPE: Research Support, Non-U.S. Gov

Journal: The Journal of cell biology

VOLUME: 179

Page Numbers: 485-500

Journal Abbreviation: J. Cell Biol.

ISSN: 0021-9525

DAY: 5

MONTH: Nov

YEAR: 2007

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

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LANGUAGE: eng

NlmUniqueID: 375356

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Grant and Affiliation Information for Functional multivesicular bodies are required for autophagic clearance of protein aggregates associated with neurodegenerative disease.

AFFILIATION: Centre for Cancer Biomedicine, University of Oslo and Department of Biochemistry, The Norwegian Radium Hospital, Montebello, N-0310 Oslo, Norway.

Country: United States

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MEDLINETA: J Cell Biol

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