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Article

Accumulation of wildtype and ALS-linked mutated VAPB impairs activity of the proteasome.

PloS one - 1 Jan 2011

Moumen Anice, Virard Isabelle, Raoul Cédric

Abstract excerpt

Cellular homeostasis relies on a tight control of protein synthesis, folding and degradation, in which the endoplasmic reticulum (ER) quality control and the ubiquitin proteasome system (UPS) have an instrumental function. ER stress and aberrant accumulation of misfolded proteins represent a pathological signature of amyotrophic lateral sclerosis (ALS), a fatal paralytic disorder caused by the selective...

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Share to Endoplasmic Reticulum Stress
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Methodology Data sources Editorial policy Privacy About Contact Helena Bioinformatics

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Source

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Identifiers

PMID: 21998752
DOI: 10.1371/journal.pone.0026066
PMCID: PMC3187839

Genes

VAPB

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 3 Sept 2026.

Related research

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Accumulation of wildtype and ALS-linked mutated VAPB impairs activity of the proteasome.PMID 21998752Read ↗

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Restructured endoplasmic reticulum generated by mutant amyotrophic lateral sclerosis-linked VAPB is cleared by the proteasomeSemantic proximity Read ↗

Amyotrophic lateral sclerosis (ALS)-associated VAPB-P56S inclusions represent an ER quality control compartment.Semantic proximity Read ↗

VAPB Confers Selective Neuroprotection by Driving Autophagic Degradation of Pathogenic Aggregates in ALSSemantic proximity Read ↗

Mutant VAPB: Culprit or Innocent Bystander of Amyotrophic Lateral Sclerosis?Semantic proximity Read ↗

Characterization of the Properties of a Novel Mutation in VAPB in Familial Amyotrophic Lateral SclerosisSemantic proximity Read ↗

A role for mitochondria-ER crosstalk in amyotrophic lateral sclerosis 8 pathogenesis.Semantic proximity Read ↗

The Link between VAPB Loss of Function and Amyotrophic Lateral Sclerosis.Semantic proximity Read ↗

A VAPB mutant linked to amyotrophic lateral sclerosis generates a novel form of organized smooth endoplasmic reticulum.Semantic proximity Read ↗

A mutation in human VAP-B--MSP domain, present in ALS patients, affects the interaction with other cellular proteins.Semantic proximity Read ↗

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