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Article

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

FASEB journal : official publication of the Federation of American Societies for Experimental Biology - 1 May 2010

Fasana Elisa, Fossati Matteo, Ruggiano Annamaria, Brambillasca Silvia, Hoogenraad Casper C, Navone Francesca, Francolini Maura, Borgese Nica

Abstract excerpt

VAPB (vesicle-associated membrane protein-associated protein B) is an endoplasmic reticulum (ER)-resident tail-anchored adaptor protein involved in lipid transport. A dominantly inherited mutant, P56S-VAPB, causes a familial form of amyotrophic lateral sclerosis (ALS) and forms poorly characterized inclusion bodies in cultured cells. To provide a cell biological basis for the understanding of mutant VAPB...

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Identifiers

PMID: 20008544
DOI: 10.1096/fj.09-147850

Genes

VAPB

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A VAPB mutant linked to amyotrophic lateral sclerosis generates a novel form of organized smooth endoplasmic reticulum.PMID 20008544Read ↗

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 ↗

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Characterization of amyotrophic lateral sclerosis-linked P56S mutation of vesicle-associated membrane protein-associated protein B (VAPB/ALS8).Semantic proximity Read ↗

Motor neuron disease-associated mutant vesicle-associated membrane protein-associated protein (VAP) B recruits wild-type VAPs into endoplasmic reticulum-derived tubular aggregates.Semantic proximity Read ↗

Yeast Models of Amyotrophic Lateral Sclerosis Type 8 Mimic Phenotypes Seen in Mammalian Cells Expressing Mutant VAPBP56S.Semantic proximity Read ↗

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Proteomics-Based Approach Identifies Altered ER Domain Properties by ALS-Linked VAPB Mutation.Semantic proximity Read ↗

A mutation in VAPB that causes amyotrophic lateral sclerosis also causes a nuclear envelope defect.Semantic proximity Read ↗

Vapb/Amyotrophic lateral sclerosis 8 knock-in mice display slowly progressive motor behavior defects accompanying ER stress and autophagic responseSemantic proximity Read ↗

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