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Article

Dynamics of mutated GFAP aggregates revealed by real-time imaging of an astrocyte model of Alexander disease.

Experimental cell research - 1 Aug 2007

Mignot Cyril, Delarasse Cécile, Escaich Séverine, Della Gaspera Bruno, Noé Eric, Colucci-Guyon Emma, Babinet Charles, Pekny Milos, Vicart Patrick, Boespflug-Tanguy Odile, Dautigny André, Rodriguez Diana, Pham-Dinh Danielle

Abstract excerpt

Alexander disease (AxD) is a rare neurodegenerative disorder characterized by large cytoplasmic aggregates in astrocytes and myelin abnormalities and caused by dominant mutations in the gene encoding glial fibrillary acidic protein (GFAP), the main intermediate filament protein in astrocytes. We tested the effects of three mutations (R236H, R76H and L232P) associated with AxD in cells transiently expressing...

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Source

Identifiers

PMID: 17604020
DOI: 10.1016/j.yexcr.2007.04.035

Genes

GFAP

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PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-07.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

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Dynamics of mutated GFAP aggregates revealed by real-time imaging of an astrocyte model of Alexander disease.PMID 17604020Read ↗

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Lysosomal function, resistance to stress and repair are compromised by expression of the Alexander disease GFAP R239C mutantSemantic proximity Read ↗

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Glial fibrillary acidic protein is pathologically modified in Alexander disease.Semantic proximity Read ↗

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